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Showing posts with label BLOOD DISORDERS. Show all posts
Showing posts with label BLOOD DISORDERS. Show all posts

ANAEMIA

Anaemia is a condition in which a person's blood has a lower than normal number of red blood cells (RBCs), or the RBCs don't have enough haemoglobin.

Haemoglobin (an iron-rich protein that gives the red colour to blood) carries oxygen from the lungs to the rest of the body. In people with anaemia, the blood does not carry enough oxygen to the rest of the body. As a result, people with anaemia feel tired, along with other symptoms, because their bodies are not receiving enough oxygen. In severe or prolonged cases of anaemia, the lack of oxygen in the blood can cause serious and sometimes fatal damage to the heart and other organs of the body.

PERNICIOUS ANAEMIA

What Is Pernicious Anaemia?

Pernicious anaemia is a condition in which the body does not make enough red blood cells due to a lack of vitamin B12 in the body. It usually occurs in people whose bodies have lost the ability to absorb vitamin B12 from food.

Anaemia

Pernicious anaemia is one of many different types of anaemia. Having anaemia means you do not have enough healthy red blood cells. When a person has anaemia, the blood cannot carry enough oxygen to the cells of the body. The most common symptom of anaemia is feeling tired.

Pernicious Anaemia

In pernicious anaemia, the blood cells do not divide normally and are too large. They have trouble getting out of the bone marrow. The problem is due to a lack of vitamin B12 in the body. Vitamin B12 is one of the B vitamins; B vitamins are found in animal foods such as meat, fish, eggs, milk, and other dairy products. Vitamin B12 is necessary for the body to make red blood cells. It is also needed for the normal working of the nervous system.

People can develop low levels of this important vitamin in three main ways:

  • From the lack of a protein in the stomach that helps the body absorb vitamin B12. The protein is called intrinsic factor. Intrinsic factor is made by special cells in the lining of the stomach. In some people, these cells are destroyed by the body's immune system or as a result of stomach surgery. When this happens, intrinsic factor is not produced and vitamin B12 cannot be absorbed. This is the most common cause of vitamin B12 deficiency.
  • From not getting enough vitamin B12 in the diet. This can be the result of eating a strict vegetarian diet or a poor diet due to factors such as aging or alcoholism.
  • From certain intestinal disorders that interfere with the absorption of vitamin B12, such as Crohn's disease and intestinal infections.

The condition was named �pernicious� anaemia because it was often fatal in the years before the cause was discovered to be a lack of vitamin B12, and no specific treatments were available. Now it is easy to treat with vitamin B12 pills or injections. Pernicious anaemia can be severe if it goes on for a long time without being treated. If it is not treated, it can cause permanent damage to the body. Pernicious anaemia is especially common in older adults.

Effects of Pernicious Anaemia on the Body

People who have pernicious anaemia often feel tired and weak because the body is not getting enough oxygen. Over time, if untreated, this disease can cause serious problems for the heart, nerves, and other parts of the body.

Heart. In people with anaemia, the heart has to work harder to pump blood to get enough oxygen to the body's organs and tissues. This stress on the heart can cause heart murmurs (an extra or unusual sound heard during the heartbeat), fast or irregular heartbeats, an enlarged heart, or even heart failure.

A lack of vitamin B12 or folic acid (folate) can cause extra problems for the heart because it raises the level in the body of a chemical called homocysteine (ho-mo-SIS-teen). High levels of homocysteine add to the buildup of fatty deposits in blood vessels, which in turn can lead to heart attacks and strokes.

Nerves. A lack of vitamin B12 can damage nerve cells and cause problems such as tingling and numbness in hands and feet and problems with walking and balance. A vitamin B12 deficiency can cause changes in taste, smell, and vision. Finally, it can cause mental changes, including memory loss and confusion.

Digestive tract. A lack of vitamin B12 may change the surface of the tongue and shrink or thin the stomach lining. Any changes that occur in the stomach can put a person at risk for stomach cancer.

Outlook

Pernicious anaemia is usually easy to treat with vitamin B12 pills or shots, although some people develop permanent nerve damage before they find out they have the disease and get treatment. Since pernicious anaemia does increase the risk of developing stomach cancer, doctors may do periodic cancer tests to check for it. Overall, however, people with pernicious anaemia who get proper lifelong treatment can have a normal lifespan.

Other Names for Pernicious Anaemia

  • Megaloblastic anaemia
  • Vitamin B12 deficiency anaemia
  • Combined systems disease
  • Congenital pernicious anaemia

What Causes Pernicious Anaemia?

Major Causes

Pernicious anaemia is caused by a lack of vitamin B12 in the body. The main reason for the vitamin B12 deficiency is the loss of parietal cells in the lining of the stomach. These cells make intrinsic factor, which helps the body absorb vitamin B12 in the small intestine. In some people, the body's immune system may attack and destroy the parietal cells. Doctors don't know exactly why or how this happens, or if the immune system produces antibodies in reaction to normally aging or dying parietal cells.

As a result of this immune system attack, the stomach lining shrinks, and the parietal cells in the lining of the stomach disappear. The stomach stops producing intrinsic factor. Over time, vitamin B12 deficiency develops.

Loss of intrinsic factor can also be due to removal of the stomach lining in various kinds of stomach surgery. This surgery includes removal of all or part of the stomach as well as stomach surgery for weight loss.

There is also a rare inherited disorder in which children are born without the ability to produce intrinsic factor.

Other Causes

Less common causes of pernicious anaemia include a diet low in vitamin B12, intestinal problems, and certain medicines.

Lack of Vitamin B12 in the Diet

People can develop pernicious anaemia if they don't get enough vitamin B12 in the foods that they eat. This condition takes many years to develop because it takes time to use up the vitamin B12 already stored in the body.

Some people who are strict vegetarians can develop pernicious anaemia, especially if they do not eat meat, poultry, fish, eggs, or dairy products��the best food sources of vitamin B12. Breastfed infants of strict vegetarian mothers can develop anaemia in a short time because they don't have enough vitamin B12 stored in their bodies. They can be given vitamin B12 supplements to prevent this type of anaemia.

Some people develop pernicious anaemia because of a poor diet due to conditions such as alcoholism or aging.

Disorders of the Small Intestine

Some intestinal problems can cause poor absorption of vitamin B12. These problems include:

  • An infection caused by parasites or an overgrowth of bacteria in the intestine
  • Coeliac disease (also known as sprue), a genetic disorder that makes a person unable to tolerate gluten
  • Crohn's disease, an inflammatory bowel disease
  • Not enough stomach acid to digest food�a problem that can occur in older adults

Medicines

Long-term use of certain medicines may lead to pernicious anaemia. Examples of these are medicines that reduce acid in the stomach and certain diabetes medicines (such as metformin, phenformin, and biguanides).

Who Is At Risk for Pernicious Anaemia

Populations Affected

People of all races can develop pernicious anaemia. However, people of northern European or African descent have a higher risk than other races and ethnic groups.

Men and women in the United States are equally likely to develop the disease. It is more common in older adults than younger people, and it is rare in children.

Major Risk Factors

A person's chances of developing pernicious anaemia may be higher if he or she has:

  • A family history of pernicious anaemia (blood relatives with the disease)
  • A disorder such as diabetes or a thyroid problem
  • An intestinal disorder that keeps the body from absorbing vitamin B12 well

Pernicious anaemia is more likely to develop in people who do not eat foods high in vitamin B12 for long periods of time. This includes some vegetarians, elderly people, and people with alcoholism.

What Are the Signs and Symptoms of Pernicious Anaemia?

Major Signs and Symptoms

Major signs and symptoms of pernicious anaemia are feeling tired and weak and having a bright red, smooth tongue. Common symptoms of nerve damage caused by this disease are tingling and numbness in the hands and feet.

Symptoms most often develop slowly over time if the disease is not treated. Some people may experience mental changes and nerve problems before blood tests show that they have anaemia. This is more likely to happen in older adults than in younger people.

Other Signs and Symptoms

Other signs and symptoms of pernicious anaemia may include pale or yellowish skin, a low-grade fever, and dizziness when standing up. Infants with the condition may show unusual movements or a delayed development and failure to thrive.

Signs and Symptoms of Complications Associated With Pernicious Anaemia

Complications seen with pernicious anaemia can involve the heart, nerves and brain, and digestive tract. Some of the complications are due to the anaemia; others are the effect of a low vitamin B12 level on parts of the body.

Heart

Signs and symptoms of heart problems may include shortness of breath and chest pain. Heart murmurs, a rapid heart rate, and heart failure can develop.

Nerves and Brain

In addition to tingling or numbness in the hands and feet, signs and symptoms of problems with the nerves may include difficulty walking, unsteady movement, and loss of balance. There can be changes in vision, taste, and smell. Memory loss, confusion, depression, and even psychosis can develop.

Digestive Tract

Signs and symptoms of untreated pernicious anaemia can occur all along the digestive track. They can start with a bright red, smooth tongue and may include mouth sores or bleeding gums. The liver could be enlarged. Nausea and vomiting may occur, along with a sense of fullness, gas, or heartburn. Changes in bowel habits could include constipation or diarrhoea. A person might have a loss of appetite or weight loss.

How Is Pernicious Anaemia Diagnosed?

Pernicious anaemia is diagnosed using a person's medical history, physical exam, and tests that can determine the type and cause of anaemia. A doctor can use these methods to find out how severe the problem is, its cause, and the appropriate treatment. Mild to moderate anaemia may have no signs or symptoms. In fact, anaemia is often discovered unexpectedly on screening tests.

Specialists Involved

Primary care doctors, such as a family doctor, often diagnose and treat pernicious anaemia. Other kinds of doctors may also be involved, including:

  • A neurologist (nervous system specialist)
  • A cardiologist (heart specialist)
  • A haematologist (blood disease specialist)
  • A gastroenterologist (digestive tract disease specialist)

Medical and Family History

Your doctor may ask detailed questions about many symptoms, including feeling tired and weak and others listed in the section What Are the Signs and Symptoms of Pernicious Anaemia? The doctor may ask about any personal or family history of anaemia, diabetes, or diseases of the immune system. You may be asked about any surgery you have had, especially stomach surgery. The doctor may also ask you about your diet and about the medicines you are taking.

Physical Exam

A physical exam may include:

  • Checking for pale or yellowish skin and a red, smooth tongue
  • Listening to the heart to check for a rapid heartbeat or murmur
  • Feeling the abdomen to check the size of the liver

Your doctor will also order a number of tests or procedures to be sure about the type of anaemia you have and how severe it is.

Diagnostic Tests and Procedures

Complete Blood Count

Usually, the first test used to diagnose anaemia is a complete blood count (CBC). The CBC tells a number of things about a person's blood, including:

  • The haemoglobin level. Haemoglobin is the iron-rich protein in red blood cells that carries oxygen through the body. A low haemoglobin level means a person has anaemia.
  • The haematocrit level. The haematocrit level measures how much of the blood is made up of red blood cells. Low haematocrit is another sign of anaemia.

The CBC also checks:

  • The number of red blood cells. Too few red blood cells means a person has anaemia. A low number of red blood cells is usually seen with either a low haemoglobin or a low haematocrit level, or both.
  • The number of white blood cells. White blood cells are involved in fighting infection.
  • The number of platelets in the blood. Platelets are small cells that are involved in blood clotting.
  • Red blood cell size. The mean cell volume is the name of a test that measures the average size (volume) of red blood cells. In pernicious anaemia, the red blood cells are usually larger than normal. This is called macrocytosis (MAK-ro-si-TO-sis).

Tests To Check the Vitamin B12 Level

  • Vitamin B12. The level of vitamin B12 in the bloodstream may be normal or borderline even when the total amount of B12 in the body is low.
  • Folic acid (folate). This is another B vitamin that can be low when the B12 level is low. A lack of folic acid can also cause anaemia.
  • Homocysteine. Homocysteine is high in anaemia due to the lack of vitamin B12 or folic acid. Folate deficiency is more common because this vitamin is used up more quickly and the dietary need is greater. In this case, the blood's B12 level can be normal.
  • Methylmalonic acid. The level of methylmalonic acid is high in anaemia due to a lack of vitamin B12 or folic acid. Methylmalonic acid can also be checked with a urine test.

Other Blood Tests

Other blood tests check for:

  • The presence of intrinsic factor antibodies and parietal cell antibodies. These antibodies in the blood may mean that they are destroying the intrinsic factor or parietal cells.
  • Levels of bilirubin, potassium, or cholesterol in the blood.
  • Serum iron and iron binding capacity.
  • The number of reticulocytes. Reticulocytes are young, red blood cells. The reticulocyte test is used to see if the bone marrow is producing red blood cells at the proper rate. A lower than average number of reticulocytes can mean that the bone marrow is not making enough red blood cells. The reticulocyte number is low in people with pernicious anaemia.

Schilling Test

The Schilling test is a urine test that measures how well the body absorbs vitamin B12. It is not used as much now as it was in the past.

Bone Marrow Tests

In some cases, a doctor may want to do a bone marrow biopsy or aspiration. A bone marrow biopsy is a minor surgical procedure to remove a small amount of bone marrow tissue. In a bone marrow aspiration, the doctor removes a small amount of bone marrow fluid through a needle. Bone marrow biopsy or aspiration tests whether the bone marrow is healthy and can show whether the bone marrow is making enough blood cells.

How Is Pernicious Anaemia Treated?

Doctors treat pernicious anaemia by replacing the missing vitamin B12 in the body. People who have pernicious anaemia need treatment, usually for the rest of their lives. Without treatment, pernicious anaemia can cause serious problems and can even be fatal.

Goals of Treatment

The goals of treating pernicious anaemia are to:

  • Stop the anaemia and symptoms through vitamin B12 treatment
  • Prevent complications, such as heart or nerve damage
  • Provide ongoing followup to make sure that the treatment is working
  • Treat the underlying cause, if one can be found

Specific Types of Treatment

Fortunately, pernicious anaemia is usually easy to treat with either vitamin shots (injections) or pills. Symptoms may begin to improve within a few days after the start of treatment.

  • Vitamin B12 shots. People with pernicious anaemia may get daily or weekly shots at first, then one shot every month. Some people get vitamin B12 shots and also take vitamin B12 pills.
  • Vitamin B12 pills. Many people with pernicious anaemia can be treated successfully with vitamin B12 pills. Often, the pills work as well as the shots. Because only a small amount of vitamin B12 is absorbed by the body, high doses are given.

Vitamin B12 can also be given in a gel or spray for the nose.

Treatment for the underlying causes of vitamin B12 deficiency may be needed. To help the body absorb vitamin B12, for example, a person might need antibiotics to treat stomach infections or surgery to treat intestinal problems. If the vitamin B12 level is due to a poor diet, then a person can learn how to correct the diet.

The doctor may also recommend limiting physical activity until anaemia symptoms have improved.

How Can Pernicious Anaemia Be Prevented?

Doctors do not know how to prevent pernicious anaemia that occurs from the immune system destroying parietal cells in the stomach. The most common cause of pernicious anaemia is the loss of stomach cells that make intrinsic factor.

Pernicious anaemia due to a diet low in vitamin B12 is not common. But some people who are strict vegetarians or who have a poor diet for a long time can develop this condition. Eating foods high in vitamin B12 and folic acid can help prevent low vitamin B12 levels. Some of these foods are:

  • Eggs, meat, poultry, or shellfish
  • Milk, orange juice, or oranges
  • Fortified cereals, wheat germ, rice, or barley
  • Romaine lettuce, spinach, and other green leafy vegetables
  • Sprouts, broccoli, asparagus
  • Peas, peanuts, beans, lentils, soy beans, and chickpeas

Vitamin B12 also can be found in multivitamins and in B-complex vitamin supplements.

Doctors may recommend supplements for people at risk of developing vitamin B12 deficiency, such as infants and children of strict vegetarians.

Living With Pernicious Anaemia

People treated for pernicious anaemia can recover, feel well, and live normal lives, although they must be sure to receive enough vitamin B12 throughout their lives. If a person has developed health problems caused by pernicious anaemia, such as nerve damage, treatment may reverse the damage.

Ongoing Health Care Needs

People with pernicious anaemia usually need to see a doctor regularly for checkups and ongoing treatment with vitamin B12. If you are being treated for pernicious anaemia, you will need to take vitamin B12 supplements as directed by your doctor to prevent the return of symptoms.

Visits to the doctor will focus on monitoring for signs of vitamin B12 deficiency in your body, making treatment changes as needed, and checking for the possible development of stomach cancer.

Doctor visits will also focus on the foods that you eat and whether you are eating enough foods that contain vitamin B12. A paediatrician may prescribe vitamin B12 supplements for infants and children of strict vegetarians.

Continued treatment may be needed for any ongoing problems due to nerve damage.

Family Members

If you have been diagnosed with pernicious anaemia, you should tell your family members about the disease�especially your children and your siblings. Because pernicious anaemia runs in families, they may be more likely to develop the disease.

Key Points

  • Pernicious anaemia is a condition caused by too little vitamin B12 in the body. It is also called vitamin B12 deficiency anaemia.
  • Vitamin B12 helps the body make healthy red blood cells and helps keep nerve cells healthy. It is found in animal foods, including meat, fish, eggs, milk, and other dairy products.
  • The most common cause of pernicious anaemia is the loss of stomach cells that make intrinsic factor. Intrinsic factor helps the body absorb vitamin B12 in the intestine. The loss of parietal cells may be due to destruction by the body's own immune system.
  • Pernicious anaemia can cause permanent damage to nerves and other organs if it goes on for a long time without being treated. It also raises the risk for developing stomach cancer.
  • Common signs and symptoms of pernicious anaemia are:
    • Feeling tired and weak
    • Tingling and numbness in hands and feet
    • A bright red, smooth tongue
  • Pernicious anaemia is diagnosed using family history and medical history, a physical exam, and diagnostic tests and procedures.
  • Pernicious anaemia is easy to treat with vitamin B12 pills or shots as well as diet changes. Lifelong treatment is needed.
  • Complications caused by untreated pernicious anaemia may be reversible with treatment.
  • Doctors don't know how to prevent pernicious anaemia that is caused by the immune system destroying stomach cells.
  • Eating foods high in vitamin B12 and folic acid can help prevent vitamin B12 deficiency caused by a poor diet.

OEDEMA

Oedema is swelling of any organ or tissue due to accumulation of excess lymph fluid, without an increase of the number of cells in the affected tissue. Oedema can accumulate in almost any location in the body, but the most common sites are the feet and ankles.

Oedema is the increase of interstitial fluid in any organ. Generally, the amount of interstitial fluid is in the balance of homeostasis. Increased secretion of fluid into the interstitium or impaired removal of this fluid may cause oedema.

Generation of interstitial fluid is regulated by the Starling equation of tissue fluid which states that it depends on the balance of osmotic pressure and of hydrostatic pressure which act in opposite directions across the semipermeable capillary walls. Consequently, anything that increases oncotic pressure outside blood vessels (for example inflammation), or reduces oncotic pressure in the blood (states of low plasma osmolality, for example cirrhosis) will cause oedema. Increased hydrostatic pressure inside the blood vessel (for example in heart failure) will have the same effect. If the permeability of the capillary walls increases, more fluid will tend to escape out of the capillary, as can happen when there is inflammation.

Abnormal removal of interstitial fluid is caused by obstruction of the lymphatic system, for example due to pressure from a cancer or enlarged lymph nodes, destruction of lymph vessels by radiotherapy, or infiltration of the lymphatics by infection such as elephantiasis.

Peripheral oedema

Oedema without a modifier usually refers to peripheral or dependent oedema, the accumulation of fluid in the parts of the body that are most affected by gravity. In ambulatory people these are the legs, although in those who are bedbound the first manifestation may be sacral oedema. If severe enough, peripheral oedema may progress to involve the abdominal or even thoracic wall (this may be referred to as generalized oedema or anasarca). In particular oedema states (e.g. nephrotic syndrome, see below), periorbital oedema (around the eyes) may be present.

Some phenomena may distinguish different causes of peripheral oedema. Most peripheral oedema is pitting oedema - pressing down will lead to a shift in the interstitial fluid and the formation of a small pit that resolves over seconds. Non-pitting oedema may reflect lymphedema, a form of oedema that develops when the lymph vessels are obstructed.

Causes of peripheral oedema are:

  • high hydrostatic pressure of the veins, leading to poor reabsorption of fluid
  • o venous obstruction, e.g. deep vein thrombosis (typically one-sided)
    o congestive heart failure
    o varicose veins
    o asymmetric compression of thigh and leg (e.g., knee pads, tight jeans)
    • low oncotic pressure
    • o cirrhosis
      o malnutrition
      o nephrotic syndrome (renal protein loss)
      o epidemic dropsy
      • obstruction of lymph drainage
      • o infection
        o cancer
        o fibrosis after surgery
        o filariasis
        • inflammation (active secretion of fluid into the interstitial space due to increased membrane permeability by inflammatory mediators):
        • o allergic conditions (e.g. angioedema)
          o any other form of inflammation (tumour - or swelling - is one of the main characteristics of inflammation)

          Organ-specific oedema

          Oedema of specific organs (cerebral oedema, pulmonary oedema, macular oedema) may also occur, each with different specific causes to peripheral oedema, but all based on the same principles. Ascites is effectively oedema within the peritoneal cavity, as pleural effusions are effectively oedema in the pleural cavity. Causes of oedema which are generalised to the whole body can cause oedema in multiple organs and peripherally. For example, severe heart failure can cause peripheral oedema, pulmonary oedema, pleural effusions and ascites.

Source: wikipedia GFDL

MYELODYSPLASTIC SYNDROME

  • Myelodysplastic syndromes are a group of diseases in which the bone marrow does not make enough healthy blood cells.
  • There are several types of myelodysplastic syndromes.
  • Age and past treatment with chemotherapy or radiation therapy affect the risk of developing a myelodysplastic syndrome.
  • Possible signs of myelodysplastic syndrome include feeling tired and shortness of breath.
  • Tests that examine the blood and bone marrow are used to detect (find) and diagnose myelodysplastic syndromes.
  • Myelodysplastic syndromes are diagnosed based on certain changes in the blood cells and bone marrow.
  • Certain factors affect prognosis (chance of recovery) and treatment options.

Myelodysplastic syndromes are a group of diseases in which the bone marrow does not make enough healthy blood cells.

Myelodysplastic syndromes are diseases of the blood and bone marrow. Normally, the bone marrow produces stem cells (immature cells) that develop into mature blood cells. There are 3 types of mature blood cells:

  • Red blood cells that carry oxygen and other materials to all tissues of the body.
  • White blood cells that fight infection and disease.
  • Platelets that help prevent bleeding by causing blood clots to form.

In myelodysplastic syndromes, the stem cells do not mature into healthy red blood cells, white blood cells, or platelets. The immature blood cells, called blasts, do not function normally and either die in the bone marrow or soon after they enter the blood. This leaves less room for healthy white blood cells, red blood cells, and platelets to develop in the bone marrow. When there are fewer blood cells, infection, anemia, or easy bleeding may occur.

There are several types of myelodysplastic syndromes.

Myelodysplastic syndromes have too few of one or more types of healthy blood cells in the bone marrow or blood. Myelodysplastic syndromes include the following diseases:

  • Refractory anemia.
  • Refractory anemia with ringed sideroblasts.
  • Refractory anemia with excess blasts.
  • Refractory anemia with excess blasts in transformation.
  • Refractory cytopenia with multilineage dysplasia.
  • Myelodysplastic syndrome associated with an isolated del(5q) chromosome abnormality.
  • Unclassifiable myelodysplastic syndrome.

Refer to the following summaries for information about other blood cell diseases:

  • Myelodysplastic/Myeloproliferative Disease Treatment
  • Chronic Myeloproliferative Disorders Treatment

Age and past treatment with chemotherapy or radiation therapy affect the risk of developing a myelodysplastic syndrome.

Risk factors include the following:

  • Being older than 60 years.
  • Past treatment with chemotherapy or radiation therapy.
  • Being exposed to certain chemicals, including tobacco smoke, pesticides, and solvents such as benzene.

Possible signs of myelodysplastic syndrome include feeling tired and shortness of breath.

Myelodysplastic syndromes often do not cause early symptoms and are sometimes found during a routine blood test. Other conditions may cause the same symptoms. A doctor should be consulted if any of the following problems occur:

  • Shortness of breath.
  • Weakness or feeling tired.
  • Having skin that is paler than usual.
  • Easy bruising or bleeding.
  • Petechiae (flat, pinpoint spots under the skin caused by bleeding).
  • Fever or frequent infections.

Tests that examine the blood and bone marrow are used to detect (find) and diagnose myelodysplastic syndromes.

The following tests and procedures may be used:

  • Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.
  • Complete blood count (CBC) with differential: A procedure in which a sample of blood is drawn and checked for the following:
  • o The number of red blood cells and platelets.
    o The number and type of white blood cells.
    o The amount of haemoglobin (the protein that carries oxygen) in the red blood cells.
    o The portion of the blood sample made up of red blood cells.
  • Peripheral blood smear: A procedure in which a sample of blood is checked for changes in the number, type, shape, and size of blood cells and for too much iron in the red blood cells.
  • Cytogenetic analysis: A test in which cells in a sample of blood or bone marrow are viewed under a microscope to look for certain changes in the chromosomes.
  • Bone marrow aspiration and biopsy: The removal of a small piece of bone and bone marrow by inserting a needle into the hipbone or breastbone. A pathologist views both the bone and bone marrow samples under a microscope to look for abnormal cells.

Myelodysplastic syndromes are diagnosed based on certain changes in the blood cells and bone marrow.

  • Refractory anemia: There are too few red blood cells in the blood and the patient has anemia. The number of white blood cells and platelets is normal.
  • Refractory anemia with ringed sideroblasts: There are too few red blood cells in the blood and the patient has anemia. The red blood cells have too much iron. The number of white blood cells and platelets is normal.
  • Refractory anemia with excess blasts: There are too few red blood cells in the blood and the patient has anemia. Five percent to 19% of the cells in the bone marrow are blasts and there are a normal number of blasts found in the blood. There also may be changes to the white blood cells and platelets. Refractory anemia with excess blasts may progress to acute myeloid leukaemia. Refer to the Adult Acute Myeloid Leukaemia Treatment summary for more information.
  • Refractory anemia with excess blasts in transformation: There are too few red blood cells, white blood cells, and platelets in the blood and the patient has anemia. Twenty percent to 30% of the cells in the bone marrow are blasts and more than 5% of the cells in the blood are blasts. Refractory anemia with excess blasts in transformation is sometimes called acute myeloid leukaemia.
  • Refractory cytopenia with multilineage dysplasia: There are too few of at least two types of blood cells. Less than 5% of the cells in the bone marrow are blasts and less than 1% of the cells in the blood are blasts. If red blood cells are affected, they may have extra iron. Refractory cytopenia may progress to acute leukaemia.
  • Myelodysplastic syndrome associated with an isolated del(5q) chromosome abnormality: There are too few red blood cells in the blood and the patient has anemia. Less than 5% of the cells in the bone marrow and blood are blasts. There is a specific change in the chromosome.
  • Unclassifiable myelodysplastic syndrome: There are too few of one type of blood cell in the blood. The number of blasts in the bone marrow and blood is normal, and the disease is not one of the other myelodysplastic syndromes.

Certain factors affect prognosis (chance of recovery) and treatment options.

The prognosis (chance of recovery) depends on the following:

  • Whether the myelodysplastic syndrome occurred after chemotherapy or radiation therapy for another disease.
  • The number of blast cells in the bone marrow.
  • Whether one or more types of blood cells are affected.
  • Certain changes in the chromosomes.

Treatment options depend on the following:

  • Whether the myelodysplastic syndrome occurred after chemotherapy or radiation therapy for another disease.
  • Whether the myelodysplastic syndrome has progressed after being treated.
  • The age and general health of the patient.

Stages of Myelodysplastic Syndromes

There is no staging system for myelodysplastic syndromes. Treatment is based on whether the disease developed after the patient was exposed to factors that cause myelodysplastic syndrome or whether the disease was previously treated. Myelodysplastic syndromes are grouped for treatment as follows:

De novo myelodysplastic syndromes

De novo myelodysplastic syndromes develop without any known cause.

Secondary myelodysplastic syndromes

Secondary myelodysplastic syndromes develop after the patient was treated with chemotherapy or radiation therapy for other diseases or after being exposed to radiation or certain chemicals that are linked to the development of myelodysplastic syndromes. Secondary myelodysplastic syndromes may be harder to treat than de novo myelodysplastic syndromes.

Previously treated myelodysplastic syndromes

The myelodysplastic syndrome has been treated but has not gotten better.

Treatment Option Overview

Key Points for This Section

  • There are different types of treatment for patients with myelodysplastic syndromes.
  • The usual goal of treatment for myelodysplastic syndromes is to prevent symptoms and related problems.
  • Three types of standard treatment are used:
  • o Chemotherapy
    o Supportive care
    o Chemotherapy with stem cell transplant

    There are different types of treatment for patients with myelodysplastic syndromes.

    Different types of treatments are available for patients with myelodysplastic syndromes. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. Before starting treatment, patients may want to think about taking part in a clinical trial. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with a myelodysplastic syndrome. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment.

    Clinical trials are taking place in many parts of the country. Information about ongoing clinical trials is available from the NCI Web site. Choosing the most appropriate treatment is a decision that ideally involves the patient, family, and health care team.

    The usual goal of treatment for myelodysplastic syndromes is to prevent symptoms and related problems.

    At this time, there is no treatment that will cure myelodysplastic syndrome or stop it from becoming acute leukaemia. The usual treatment is supportive care, which helps many patients manage the disease. The main goal of treatment is to prevent symptoms and problems caused by low blood cell counts, such as fatigue and infections. If the disease is severe or gets worse quickly, a more aggressive treatment may be given, such as chemotherapy to kill the blast cells. Side effects of chemotherapy may cause serious health problems and many patients do better with standard supportive care.

    Three types of standard treatment are used:

    Chemotherapy

    Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the spinal column, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated.

    Supportive care

    Supportive care is given to lessen the problems caused by the disease or its treatment. Supportive care may include the following:

    • Transfusion therapy

    Transfusion therapy (blood transfusion) is a method of giving red blood cells, white blood cells, or platelets to replace blood cells destroyed by disease or treatment. Patients who receive frequent red blood cell transfusions may have their tissues and organs damaged from the build up of extra iron. Iron chelation therapy is a treatment that uses drugs that attach to the extra iron. The drug and the iron are removed from the body in the urine.

    Platelet transfusions are usually given when the patient is bleeding or is having a procedure that may cause bleeding.

    • Drug therapy

    Erythropoietin may be given to increase the number of red blood cells and lessen the effects of anemia. Sometimes granulocyte colony-stimulating factor (G-CSF) is given with erythropoietin to help the treatment work better.

    Anticancer drugs may also be used.

    Antibiotics may be given to fight infections.

    Chemotherapy with stem cell transplant

    Stem cell transplant is a method of giving chemotherapy and replacing blood-forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of a donor and are frozen for storage. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells.

    Treatment Options for Myelodysplastic Syndromes

    De Novo Myelodysplastic Syndromes

    Treatment of de novo myelodysplastic syndromes may include the following:

    • Supportive care.
    • Chemotherapy.
    • Stem cell transplant using stem cells from a donor.
    • A clinical trial of a new anticancer drug.
    • A clinical trial of low-dose chemotherapy with stem cell transplant using stem cells from a donor.

    Secondary Myelodysplastic Syndromes

    Treatment of secondary myelodysplastic syndromes may include the following:

    • Supportive care.
    • Chemotherapy.
    • Stem cell transplant using stem cells from a donor.
    • A clinical trial of a new anticancer drug.
    • A clinical trial of low-dose chemotherapy with stem cell transplant using stem cells from a donor.

    Previously Treated Myelodysplastic Syndromes

    Treatment of previously treated myelodysplastic syndromes may include the following:

    • Supportive care.
    • A clinical trial of a new treatment, such as stem cell transplant.

LEUKAEMIA

Overview

Leukaemia is a cancer of the blood cells which are made in the bone marrow. There are several types of leukaemia, grouped as acute or chronic.

  • Gender and age may affect the risk of developing certain types of leukaemia.
  • Possible signs of leukaemia include tiredness, fever, easy bleeding or bruising.
  • Tests that examine the blood and bone marrow are used to detect and diagnose leukaemia.
  • Certain factors affect treatment options and prognosis (chance of recovery).

Types of leukaemia

Acute lymphoblastic leukaemia (ALL) is a type of cancer in which the bone marrow makes too many lymphocytes (a type of white blood cell).

Acute myeloid leukaemia (AML) is a type of cancer in which the bone marrow makes abnormal myeloblasts (a type of white blood cell), red blood cells, or platelets.

Chronic lymphocytic leukaemia (CLL) is a type of cancer in which the bone marrow makes too many lymphocytes.

Chronic myelogenous leukaemia (CML) is a disease in which the bone marrow makes too many white blood cells.

Hairy cell leukaemia is a type of cancer in which the bone marrow makes too many lymphocytes.

IRON DEFICIENCY AND IRON DEFICIENCY ANAEMIA

Iron-deficiency anaemia is a common and easily treated condition that occurs when there is not enough iron in the body. It is the most common type of anaemia. A lack of iron in the body can come from bleeding, not eating enough foods that contain iron, or not absorbing enough iron from food that is eaten.

Anaemia

The term "anaemia" is used for a group of conditions in which the number of red blood cells in the blood is lower than normal, or the red blood cells don't have enough haemoglobin. Haemoglobin�an iron-rich protein that gives the red colour to blood�carries the oxygen from the lungs to the rest of the body. In people with anaemia, the blood does not carry enough oxygen to the rest of the body. Red blood cells also remove carbon dioxide, a waste product, from cells and carry it to the lungs to be exhaled.

Red blood cells also are called RBCs or erythrocytes. Normal red blood cells are all about the same size and look like doughnuts without a hole in the center. They are produced by the spongy marrow inside the large bones of the body. Healthy red blood cells have an average lifespan of 120 days. When they die, the iron from the haemoglobin is recycled to make new red blood cells.

There are many types of anaemia. The three major causes of anaemia are blood loss, decreased production of red blood cells, or increased destruction of red blood cells. White blood cells and platelets are the two other kinds of blood cells. White blood cells help fight infection. Platelets help blood to clot. In some kinds of anaemia, there are low amounts of all three types of blood cells. The most common symptom of all types of anaemia is feeling tired because the body is not receiving enough oxygen.

Iron-Deficiency Anaemia

In iron-deficiency anaemia, the body does not have enough iron to form haemoglobin, which means there is not enough haemoglobin to carry oxygen to the whole body. The body gets its iron from food. The main foods that contain iron are meat and shellfish as well as iron-fortified foods (that is, foods that have iron added). A steady supply of iron is needed to form haemoglobin and healthy red blood cells.

A person can have low iron levels for three reasons:

  • Blood loss, either from disease or injury
  • Not getting enough iron in the diet
  • Not being able to absorb the iron in the diet

Iron-deficiency anaemia also can develop when the body needs higher levels of iron, such as during pregnancy.

Effects of Iron-Deficiency Anaemia on the Body

Iron-deficiency anaemia can range from mild to severe. A mild case usually causes no symptoms or problems. However, a severe case can cause extreme fatigue (tiredness) and weakness. Severe iron-deficiency anaemia can lead to serious problems for young children and pregnant women, and it can affect the heart.

In young children, iron-deficiency anaemia can cause a heart murmur and delays in growth and development. It puts a child at greater risk for lead poisoning and infections, and it can cause behaviour problems.

In pregnant women, iron-deficiency anaemia can increase the risk of a premature delivery and a low-birth-weight baby.

The heart is affected when there is a lack of oxygen in the body. The heart has to work harder to get enough oxygen throughout the body. Over time, this stress on the heart can lead to a fast or irregular heartbeat, chest pain, an enlarged heart, and even heart failure.

Important General Information

A lack of iron in the body is the most common nutritional problem. Iron-deficiency anaemia is the most common form of anaemia. It is most often found in young children, pregnant women, and women of childbearing age. In fact, it affects half of all pregnant women and 1 out of 5 women of childbearing age.

Outlook

Iron-deficiency anaemia can be treated successfully. The causes of iron-deficiency anaemia can most often be treated successfully as well. However, if not treated, it can lead to severe symptoms and serious problems.

What Causes Iron-Deficiency Anaemia?

Iron-deficiency anaemia occurs when there is too little iron in the body. A person can have a low iron level for three reasons:

  • Blood loss, either from disease or injury
  • Not getting enough iron in the diet
  • Not being able to absorb the iron in the diet

Iron-deficiency anaemia also can develop when the body needs higher levels of iron, such as during pregnancy.

Loss of Iron Through Blood Loss

In general, when blood is lost, iron is lost. If the body does not have enough iron reserves to make up for the iron loss, a person will develop iron-deficiency anaemia.

Blood is lost in a number of ways. In women, iron and red blood cells are lost when bleeding occurs from very long or heavy menstrual periods as well as from childbirth. Women also can lose iron and red blood cells from slowly bleeding fibroids in the uterus.

Blood also is lost through internal bleeding. Most often this loss of blood occurs slowly and can be due to:

  • A bleeding ulcer, colon polyp, or colon cancer
  • Regular use of aspirin or other pain medicine such as nonsteroidal anti-inflammatory drugs (for example, ibuprofen and naproxen)
  • Hookworm infection
  • Urinary tract bleeding

A more rapid loss or removal of blood that can cause iron-deficiency anaemia occurs in situations such as:

  • Severe injuries
  • Surgery
  • Frequent blood drawing

Lack of Iron in the Diet

Meat, poultry, fish, eggs, dairy products, or iron-fortified foods (that is, foods that have iron added) are the best sources of iron found in food. Eating patterns that exclude these foods or food supplements may lead to iron-deficiency anaemia. For example, some vegetarians do not eat enough foods with iron. Other people get iron-deficiency anaemia because of eating poorly due to alcoholism or aging. Following a diet that has an imbalance of food groups also can lead to this type of anaemia. Examples of diets that can lead to iron-deficiency anaemia include:

  • Low-fat diets. Following a low-fat diet over a long period of time may limit sources of iron from animal foods.
  • Diets high in sugars. These types of diets are often low in iron.
  • High-fiber diets. These types of diets can slow the absorption of iron.

Infants who are fed cow's milk in the first year are at risk for iron-deficiency anaemia because cow's milk is low in iron. The same is true for infants who are breastfed after 4 months of age. These infants need iron supplements.

An Increased Need for Iron

People may need more iron at some periods in their lives. If they do not get more iron at these times, they may develop iron-deficiency anaemia. Periods of rapid growth or growth spurts in children and teens are a good example of an increased need for iron. Pregnancy also is an example. The need for iron doubles during pregnancy due to an increased blood volume, the growth of the foetus, and the blood loss that occurs during childbirth.

Inability To Absorb Enough Iron From Food

Certain factors make it hard for the body to absorb enough iron from food. These factors include:

  • Intestinal surgery or diseases of the intestine, such as Crohn's disease or coeliac disease
  • Prescription medicines that reduce acid in the stomach
  • Low levels of folate, vitamin B12, or vitamin C in the diet

How Iron-Deficiency Anaemia Develops

First, iron is lost from the body by one of the ways listed above. Usually, this happens slowly over a period of time. Most often, the person is not taking in enough iron to meet the needs of the body.

Next, the body starts to use iron that it has stored. When the stored iron is used up, new red blood cells have less haemoglobin than normal, and fewer red blood cells are produced. Finally, when the number of red cells is too low, iron-deficiency anaemia develops.

Who Is At Risk for Iron-Deficiency Anaemia?

The major risk factors for iron-deficiency anaemia are blood loss and a diet low in iron. Three of the highest risk groups are women, young children, and adults with intestinal bleeding.

Populations Affected

Women

Women who lose a lot of blood during their monthly periods are at higher risk of developing iron-deficiency anaemia. About 1 in 5 women of childbearing age has iron-deficiency anaemia.

Pregnant women need twice as much iron in their diet than women who are not pregnant. If a pregnant woman doesn't get enough iron for herself and the growing baby, she can develop iron-deficiency anaemia. About half of all pregnant women have this type of anaemia.

Young Children

Infants and toddlers 6�24 months of age need a lot of iron to grow and develop. The iron that full-term infants have stored in their bodies is used up in the first 4�6 months of life. After that, infants need to get iron from food or supplements. Premature and low-birth-weight babies are at even greater risk for iron-deficiency anaemia because they don't have as much iron stored in their bodies.

Other children at risk for anaemia are:

  • Children with poor nutrition, including low-income children
  • Children with lead in their blood
  • Infants fed cow's milk before 1 year of age
  • Breastfed infants older than 4 months who are not receiving iron-rich solid foods or iron supplements

Adults With Intestinal Bleeding

Adults who bleed in their intestinal tract are at risk for iron-deficiency anaemia. This includes people who have bleeding ulcers or colon cancer. It also includes people who use medicines that can cause intestinal bleeding (for example, aspirin).

Other Adults

Other adults who are at risk for iron-deficiency anaemia include those who are on kidney dialysis, vegetarians, and older adults who have poor diets.

What Are the Signs and Symptoms of Iron-Deficiency Anaemia?

Signs and symptoms of anaemia depend on the severity of the condition. People with mild anaemia or anaemia that has come on very slowly may have no symptoms at all. However, if the anaemia is severe, the symptoms increase and become more serious. Many of the signs and symptoms of iron-deficiency anaemia are true for all kinds of anaemia.

Major Signs and Symptoms of Anaemia

The major symptom of all types of anaemia, including iron-deficiency anaemia, is fatigue (feeling tired). Fatigue is caused by having too few red blood cells to carry oxygen to the body. This lack of oxygen in the body can cause people to feel weak or dizzy, have a headache, or even pass out when changing position (for example, standing up).

Since the heart must work harder to move the reduced amount of oxygen, signs and symptoms may include shortness of breath and chest pain. This can lead to a fast or irregular heartbeat or a heart murmur.

In anaemia, the red blood cells don't have enough haemoglobin. Common signs of lack of haemoglobin include pale skin, tongue, gums, and nail beds.

Other Signs and Symptoms of Anaemia

Other signs and symptoms of anaemia can include:

  • Cold hands and feet as well as brittle nails
  • Swelling or soreness of the tongue and cracks in the sides of the mouth
  • An enlarged spleen
  • Frequent infections

Signs and Symptoms of Iron-Deficiency Anaemia

Symptoms of iron-deficiency anaemia include unusual cravings for nonfood items such as ice, dirt, paint, or starch. This craving for nonfood items is called pica.

Another symptom of iron-deficiency anaemia is developing restless legs syndrome (RLS). RLS is a disorder that causes an uncomfortable feeling in the legs that can only be relieved by movement. Sleep is difficult for people with RLS.

In infants and young children, signs and symptoms include a poor appetite, being irritable, and a slower rate of growth and development.

Some of the signs and symptoms of iron-deficiency anaemia are related to its causes, such as blood loss. Blood loss is most often seen with very heavy or long lasting menstrual bleeding or vaginal bleeding in women after menopause. Other signs of internal bleeding are bright red blood in the stool or black, tarry-looking stools.

How Is Iron-Deficiency Anaemia Diagnosed?

Iron-deficiency anaemia is diagnosed using a person's medical history, a physical exam, and diagnostic tests and procedures. A doctor can use these methods to determine how severe the anaemia is, its cause, and appropriate treatment. Mild to moderate anaemia may have no signs or symptoms. In fact, anaemia is often discovered unexpectedly on screening tests and when doctors are checking for other problems.

Specialists Involved

Primary care doctors often diagnose and treat iron-deficiency anaemia. These doctors include paediatricians, family doctors, obstetricians, or internal medicine specialists. Other doctors may be consulted, such as experts on diseases of the blood (haematologists) or experts on diseases of the digestive system (gastroenterologists).

Medical and Family History

To find the cause of the anaemia and how severe it is, the doctor may ask detailed questions about symptoms. The doctor may ask whether the person or a family member has ever had problems with anaemia. The doctor will ask about things that may cause anaemia, including illnesses, conditions (such as pregnancy), and medicines. The doctor also may ask about the person's diet and eating habits.

Physical Exam

A physical exam may include:

  • Checking for pale or yellowish skin, gums, or nail beds
  • Listening to the heart for a rapid or irregular heartbeat
  • Listening to the lungs for rapid or uneven breathing
  • Feeling the abdomen to check the size of the liver and spleen
  • Checking for signs of bleeding, including a pelvic and rectal exam (these areas are common sources of blood loss)

The doctor also will order a number of tests or procedures to be sure about the type of anaemia and how severe it is.

Diagnostic Tests and Procedures

Your doctor may order various tests or procedures to determine the type and severity of anaemia you have. Usually, the first test used to diagnose anaemia is a complete blood count (CBC). The CBC tells a number of things about a person's blood, including:

  • The haemoglobin level. Haemoglobin is the iron-rich protein in red blood cells that carries oxygen through the body. The normal range of haemoglobin levels for the general population is 11.1�15.0 g/dL. A low haemoglobin level means a person has anaemia.
  • The haematocrit (hee-MAT-oh-crit) level. The haematocrit level measures how much of the blood is made up of red blood cells. The normal range for haematocrit levels for the general population is 32�43 percent. A low haematocrit level is another sign of anaemia.

The normal range of these levels may be lower in certain racial and ethnic populations. Your doctor can explain your individual test results.

The CBC also checks:

  • The numbers of red blood cells. Too few red blood cells means a person has anaemia. A low number of red blood cells is usually seen with either a low haemoglobin or a low haematocrit level, or both.
  • The numbers of white blood cells. White blood cells are involved in fighting infection.
  • The number of platelets in the blood. Platelets are small cells that are involved in blood clotting.
  • Red blood cell size. The mean cell volume measures the average size (volume) of red blood cells. In iron-deficiency anaemia, the red blood cells are often smaller than normal.

If the CBC results confirm that you have anaemia, your doctor may order additional tests to determine the cause, severity, and correct treatment for your condition. For example, the doctor may order a reticulocyte count. Reticulocytes are young red blood cells. This test measures the number of new red blood cells in your blood. The reticulocyte test is used to determine whether your bone marrow is producing red blood cells at the proper rate.

Tests That Measure Iron Levels in the Body

Iron is needed to make haemoglobin�the protein in red blood cells that gives them their colour and carries oxygen. Several tests can be used to check the level of iron in the blood and in the body:

  • Serum iron. This test measures the amount of iron in the blood. The level of iron in the blood can be normal even when the total amount of iron in the body is low. For this reason, other iron tests are done.
  • Serum ferritin. Ferritin is a protein that helps store iron in the body. Results of this test give doctors a good idea of how much of the body's stored iron has been used up.
  • Transferrin level or total iron-binding capacity. Transferrin is a protein that carries iron in the blood. Total iron-binding capacity measures how much of the transferrin in the blood is not carrying iron. People with iron-deficiency anaemia have a high level of transferrin that has no iron.
  • Other blood tests. Other tests the doctor may order include tests that check hormone levels, especially the thyroid hormone. Blood tests also may be ordered to check the level of a chemical used by the body to make haemoglobin. It is called erythrocyte protoporphyrin.

Tests That Diagnose Gastrointestinal Bleeding

If your doctor suspects anaemia because of internal bleeding in the stomach or intestines, several tests may be used to discover the source of the bleeding.

One of the first tests ordered is the faecal occult blood test. This test checks the stool for signs of blood. It can detect even small amounts of bleeding anywhere in the intestines. If blood is found in the stool, further tests may be used to find the source of the bleeding, including:

  • Colonoscopy. In this test, a thin, flexible tube attached to a video camera is used to examine the rectum and colon for sources of bleeding.
  • Upper GI endoscopy. In this test, a thin, flexible tube attached to a video camera is used to examine the stomach and upper intestines. The doctor looks for signs of bleeding.
  • Pelvic ultrasound. This test uses sound waves to look at the uterus and other pelvic organs. It checks for causes of heavy vaginal bleeding, such as fibroids.

How Is Iron-Deficiency Anaemia Treated?

Goals of Treatment

The goals of treating iron-deficiency anaemia are to restore normal levels of red blood cells, haemoglobin, and iron as well as to treat the condition causing the anaemia.

Specific Types of Treatment

Treatment for iron-deficiency anaemia is based on the cause and the severity of the condition. It will include treatment to stop any bleeding, as well as changes in diet and iron supplements as needed. Severe anaemia may require more emergency measures.

Treatment To Stop Bleeding

Treatment will depend on why the body is bleeding and where it is bleeding. Anaemia will not improve until the bleeding is stopped.

Treatment To Increase Iron in the Diet

Your doctor may recommend a diet rich in iron, folic acid, and vitamin C to treat the anaemia. Iron in meats is more easily absorbed by the body than iron in vegetables and other foods. The best source of iron is red meat, especially beef and liver. Chicken, turkey, pork, fish, and shellfish also are good sources of iron.

Other foods high in iron are:

  • Eggs
  • Cereals, breads, or pastas that are fortified with iron
  • Beans and nuts, including peanut butter, almonds, peas, lentils, and white, red, and baked beans
  • Dried fruits (for example, raisins, apricots, and peaches), prune juice
  • Vegetables such as spinach and other dark green, leafy vegetables
  • Iron-fortified infant formula and cereals

Sources of vitamin C in foods include many fruits and vegetables such as:

  • Citrus fruits (for example, oranges, grapefruits, and lemons) and their juices
  • Kiwi fruit, mangos, apricots, strawberries, cantaloupes, and watermelons
  • Broccoli, peppers, tomatoes, cabbage, potatoes, and leafy greens (for example, romaine lettuce, turnip greens, spinach)

The doctor may prescribe supplements to treat anaemia. Supplements can correct low iron levels within months if taken as ordered. They include iron supplements in pill form and vitamin C to help the body absorb the iron. Iron supplements also come in drops for children. But iron supplements are very dangerous if taken in overdose, so it is important to keep them away from children.

Iron and vitamin C supplements can cause side effects, including dark stools and stomach irritation or heart burn. Iron also can cause constipation, and a stool softener may be needed.

Treatment for Severe and Life-Threatening Anaemia

Severe anaemia may need to be treated with hospitalization, blood transfusions, and iron injections.

How Can Iron-Deficiency Anaemia Be Prevented?

Eating a well-balanced diet rich in iron and vitamins can help prevent iron-deficiency anaemia. Red meat is the best source of iron, but other meats, including poultry and seafood, are good sources of iron as well. Besides meat, foods high in iron are:

  • Eggs
  • Cereals, breads, or pastas that are fortified with iron
  • Beans and nuts, including peanut butter, almonds, peas, lentils, and white, red, and baked beans
  • Dried fruits (for example, raisins, apricots, and peaches), prune juice
  • Vegetables such as spinach and other dark green, leafy vegetables
  • Iron-fortified infant formula and cereals

Food fads and dieting can sometimes lead to iron deficiency. Weight loss diets that stress low-fat foods can mean that a person will avoid animal foods that are good sources of iron. High-fiber diets can make it hard for iron to be absorbed. High-sugar diets are often low in iron.

Adults who eat a balanced diet usually don't need iron supplements. However, people who don't absorb iron well and those who are strict vegetarians may need them.

Preventing Anaemia in Infants and Young Children

Anaemia can be prevented in infants and young children by testing, especially in the following three age groups:

  • Premature and low-birth-weight babies less than 6 months of age
  • Babies who are 9�12 months of age
  • Babies who are 15�18 months of age

Infants absorb iron best from breast milk. They can absorb more than 50 percent of the iron in breast milk but only about 12 percent of the iron in infant formula.

Doctors usually recommend not giving cow's milk to babies for the first year. Cow's milk is low in iron. The doctor may suggest limiting cow's milk for children up to age 3 to no more than 24 ounces a day�about three full baby bottles each day. A child who is drinking a lot of milk may not be eating other foods that are better sources of iron. Drinking a lot of milk also can lead to bleeding in the intestines.

Babies need more iron as they grow and begin to eat solid foods. To help them get enough iron:

  • Infants under age 1 who are not breastfed or who are partially breastfed can be given iron-fortified infant formula. Iron fortified means that each liter of formula has 4�12 milligrams of iron.
  • Babies older than 4 months can be given iron-rich or iron-fortified solid foods such as cereal.

The child's doctor can give advice on the best diet for the infant. The doctor may recommend iron drops if the child needs an iron supplement. Giving a child too much iron can be dangerous, so it is important to be careful and follow the doctor's instructions. Parents and caregivers should keep all iron supplements and vitamins away from children. They should ask for child-proof packages for supplements.

Preventing Anaemia in Adolescents and Women of Childbearing Age

Teenaged girls and women of childbearing age are at higher risk for iron-deficiency anaemia due to blood loss from menstrual bleeding. They should be tested for anaemia every 5�10 years starting in their teens. Girls and women at higher risk for anaemia should be checked yearly. This includes women who have a history of anaemia, do not eat foods high in iron, or have heavy blood loss from menstruation or other causes.

Preventing Anaemia in Pregnant Women

Half of all pregnant women develop iron-deficiency anaemia because their volume of blood increases and because the growing foetus needs iron. Anaemia during pregnancy can lead to an increased risk of premature delivery and a low-birth-weight baby.

To prevent these problems, pregnant women need twice as much iron as women who are not pregnant. Pregnant women can get more iron from eating more iron-rich foods, from supplements, or from both. Medical care during pregnancy should include screening for anaemia.

The doctor giving prenatal care may prescribe iron supplements, which should be taken as directed. Pregnant women should notify their doctors if they have uncomfortable side effects such as constipation. The doctor also may give advice on how to get higher levels of iron through eating iron-rich foods.

Preventing Anaemia in Older Adults

Older adults may be at risk for iron deficiency due to poor diet or illnesses that reduce iron absorption. Iron deficiency can take away their sense of well-being, strength, and activeness. It also can make symptoms of other conditions worse. Doctors can advise older adults about eating iron-rich foods and how to use iron supplements to prevent iron-deficiency anaemia.

Living With Iron-Deficiency Anaemia

If you have iron-deficiency anaemia, you need to see a doctor for treatment but you can recover, feel well, and live a normal life.

Ongoing Health Care Needs

You will need regular medical checkups to make sure your iron levels are going up. At your checkups you may have changes made to your medicines or supplements, or you may get further advice on a healthy diet.

During treatment for anaemia, you may feel fatigue (tiredness) and have other symptoms until your iron levels return to normal. This can take months. Tell your doctor if you get any new symptoms or if your symptoms get worse.

Take iron supplements only with your doctor's approval. Don't decide to take them on your own. It is possible to get too high a level of iron in your body and cause a condition called iron overload.

A pregnant woman with iron-deficiency anaemia is usually tested for anaemia at 4�6 weeks after delivery, if she:

  • Was anemic during the third trimester of pregnancy
  • Lost a lot of blood during childbirth
  • Had a multiple birth (such as twins)

Key Points

  • Iron-deficiency anaemia is an illness that occurs when there is not enough iron in the body.
  • Iron helps the body make haemoglobin and healthy red blood cells. Haemoglobin is needed to carry oxygen throughout the body.
  • A person can have low iron levels for three reasons: blood loss, either from disease or injury; not getting enough iron in the diet; and not being able to absorb the iron in the diet. Iron-deficiency anaemia also can develop when the body needs higher levels of iron, such as during pregnancy.
  • One in five women of childbearing age and half of all pregnant women have iron-deficiency anaemia.
  • Infants and toddlers can be at risk for iron-deficiency anaemia.
  • The most common symptoms of iron-deficiency anaemia are fatigue (tiredness) and weakness.
  • Iron-deficiency anaemia is treated by stopping the bleeding (if the cause of the anaemia is bleeding), increasing iron in the diet, and giving iron supplements.
  • Eating a well-balanced diet rich in iron and vitamins can help prevent iron deficiency anaemia.
  • Iron-deficiency anaemia can be successfully treated.

HODGKIN'S DISEASE

Also Known as: Hodgkins's Lymphoma
  • Adult Hodgkin's lymphoma is a disease in which malignant (cancer) cells form in the lymph system.
  • There are 5 different types of Hodgkin's lymphoma. These 5 types are based on the way they look under a microscope.
  • Age, gender, and Epstein-Barr infection can affect the risk of developing adult Hodgkin's lymphoma.
  • Possible signs of adult Hodgkin's lymphoma include swollen lymph nodes, fever, night sweats, and weight loss.
  • Tests that examine the lymph nodes are used to detect (find) and diagnose adult Hodgkin's lymphoma.
  • Certain factors affect prognosis (chance of recovery) and treatment options.

What is Hodkin's Lymphoma?

Adult Hodgkin's lymphoma is a disease in which malignant (cancer) cells form in the lymph system. Hodgkin's lymphoma is also known as Hodgkin's Disease.

Adult Hodgkin's lymphoma is a type of cancer that develops in the lymph system, part of the body's immune system.

The lymph system is made up of the following:

  • Lymph: Colorless, watery fluid that travels through the lymph system and carries white blood cells called lymphocytes. Lymphocytes protect the body against infections and the growth of tumours.
  • Lymph vessels: A network of thin tubes that collect lymph from different parts of the body and return it to the bloodstream.
  • Lymph nodes: Small, bean-shaped structures that filter substances in lymph and help fight infection and disease. Lymph nodes are located along the network of lymph vessels found throughout the body. Clusters of lymph nodes are found in the underarm, pelvis, neck, abdomen, and groin.
  • Spleen: An organ that produces lymphocytes, filters the blood, stores blood cells, and destroys old blood cells. It is located on the left side of the abdomen near the stomach.
  • Thymus: An organ in which lymphocytes grow and multiply. The thymus is in the chest behind the breastbone.
  • Tonsils: Two small masses of lymph tissue at the back of the throat. The tonsils produce lymphocytes.
  • Bone marrow: The soft, spongy tissue in the center of large bones. Bone marrow produces white blood cells, red blood cells, and platelets.

Because lymph tissue is found throughout the body, Hodgkin's lymphoma can begin in almost any part of the body and spread to almost any tissue or organ in the body.

Lymphomas are divided into 2 general types: Hodgkin's lymphoma and non-Hodgkin's lymphoma. (Refer to the summary on Adult Non-Hodgkin's Lymphoma Treatment for more information.)

Hodgkin's lymphoma can occur in both adults and children; however, treatment for adults may be different than treatment for children. Hodgkin's lymphoma may also occur in patients who have acquired immunodeficiency syndrome (AIDS); these patients require special treatment. Refer to the following summaries for more information:

  • Childhood Hodgkin's Lymphoma Treatment
  • AIDS-Related Lymphoma Treatment

There are 5 different types of Hodgkin's lymphoma. These 5 types are based on the way they look under a microscope.

  • Nodular sclerosing Hodgkin's lymphoma.
  • Mixed cellularity Hodgkin's lymphoma.
  • Lymphocyte depletion Hodgkin's lymphoma.
  • Lymphocyte-rich classical Hodgkin's lymphoma.
  • Nodular lymphocyte-predominant Hodgkin's lymphoma.

Age, gender, and Epstein-Barr infection can affect the risk of developing adult Hodgkin's lymphoma.

Risk factors for adult Hodgkin's lymphoma include the following:

  • Being in young or late adulthood.
  • Being male.
  • Being infected with the Epstein-Barr virus.
  • Having a first-degree relative (parent, brother, or sister) with Hodgkin's lymphoma.

Possible signs of adult Hodgkin's lymphoma include swollen lymph nodes, fever, night sweats, and weight loss.

These and other symptoms may be caused by adult Hodgkin's lymphoma. Other conditions may cause the same symptoms. A doctor should be consulted if any of the following problems do not go away:

  • Painless, swollen lymph nodes in the neck, underarm, or groin.
  • Fever for no known reason.
  • Drenching night sweats.
  • Weight loss for no known reason.
  • Itchy skin.
  • Feeling very tired.

Tests that examine the lymph nodes are used to detect (find) and diagnose adult Hodgkin's lymphoma.

The following tests and procedures may be used:

  • Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient's past illnesses and treatments will also be taken.
  • Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following:
  • o The number of red blood cells, white blood cells, and platelets.
    o The amount of haemoglobin (the protein that carries oxygen) in the red blood cells.
    o The portion of the sample made up of red blood cells.
  • Sedimentation rate: A procedure in which a sample of blood is drawn and checked for the rate at which the red blood cells settle to the bottom of the test tube.
  • Blood chemistry studies: A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that produces it.
  • Lymph node biopsy: The removal of all or part of a lymph node. A pathologist views the tissue under a microscope to look for cancer cells. One of the following types of biopsies may be done:
  • o Excisional biopsy: The removal of an entire lymph node.
    o Incisional biopsy or core biopsy: The removal of part of a lymph node.
    o Needle biopsy or fine-needle aspiration: The removal of a sample of tissue from a lymph node with a needle.
  • Immunophenotyping: A test in which the cells in a sample of blood or bone marrow are looked at under a microscope to find out if malignant lymphocytes (cancer) began from the B lymphocytes or the T lymphocytes.

Certain factors affect prognosis (chance of recovery) and treatment options.

The prognosis (chance of recovery) and treatment options depend on the following:

  • The patient's symptoms.
  • The stage of the cancer.
  • The type of Hodgkin's lymphoma.
  • Blood test results.
  • The patient's age, gender, and general health.
  • Whether the cancer is recurrent or progressive.

Adult Hodgkin's lymphoma can usually be cured if found and treated early.

Stages of Adult Hodgkin's Lymphoma

Key Points for This Section

  • After adult Hodgkin's lymphoma has been diagnosed, tests are done to find out if cancer cells have spread within the lymph system or to other parts of the body.
  • Stages of adult Hodgkin's lymphoma may include A, B, E, and S.
  • The following stages are used for adult Hodgkin's lymphoma:
  • o Stage I
    o Stage II
    o Stage III
    o Stage IV
  • Adult Hodgkin's lymphoma may be grouped for treatment as follows:
  • o Early Favorable
    o Early Unfavorable
    o Advanced Favorable
    o Advanced Unfavorable

    After adult Hodgkin's lymphoma has been diagnosed, tests are done to find out if cancer cells have spread within the lymph system or to other parts of the body.

    The process used to find out if cancer has spread within the lymph system or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The following tests and procedures may be used in the staging process:

    • CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerised tomography, or computerised axial tomography. For adult Hodgkin's lymphoma, CT scans of the chest, abdomen, and pelvis are taken.
    • PET scan (positron emission tomography scan): A procedure to find malignant tumour cells in the body. A small amount of radionuclide glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumour cells show up brighter in the picture because they are more active and take up more glucose than normal cells do.
    • Bone marrow biopsy: The removal of a small piece of bone and bone marrow by inserting a needle into the hipbone or breastbone. A pathologist views both the bone and bone marrow samples under a microscope to look for signs of cancer.
    • Laparotomy: A surgical procedure in which an incision (cut) is made in the wall of the abdomen to check the inside of the abdomen for signs of disease. The size of the incision depends on the reason the laparotomy is being done. Sometimes organs are removed or tissue samples are taken for biopsy. This procedure is done only if it is needed to make decisions about treatment.
    • Chest x-ray: An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.
    • Needle or surgical biopsy: The removal of tissue using a thin needle or scalpel. A pathologist views the tissue under a microscope to look for cancer cells.
    • Thoracentesis: The removal of fluid from the space between the lining of the chest and the lung, using a needle. A pathologist views the fluid under a microscope to look for cancer cells.

    Stages of adult Hodgkin's lymphoma may include A, B, E, and S.

    Adult Hodgkin's lymphoma may be described as follows:

    • A: The patient has no symptoms.
    • B: The patient has symptoms such as fever, weight loss, or night sweats.
    • E: "E" stands for extranodal and means the cancer is found in an area or organ other than the lymph nodes or has spread to tissues beyond, but near, the major lymphatic areas.
    • S: "S" stands for spleen and means the cancer is found in the spleen.

    The following stages are used for adult Hodgkin's lymphoma:

    Stage I

    Stage I is divided into stage I and stage IE.

    • Stage I: Cancer is found in one lymph node group.
    • Stage IE: Cancer is found in an area or organ other than the lymph nodes.

    Stage II

    Stage II is divided into stage II and stage IIE.

    • Stage II: Cancer is found in two or more lymph node groups on the same side of the diaphragm (the thin muscle below the lungs that helps breathing and separates the chest from the abdomen).
    • Stage IIE: Cancer is found in an area or organ other than the lymph nodes and in lymph nodes near that area or organ, and may have spread to other lymph node groups on the same side of the diaphragm.

    Stage III

    Stage III is divided into stage III, stage IIIE, Stage IIIS, and stage IIIS+E.

    • Stage III: Cancer is found in lymph node groups on both sides of the diaphragm (the thin muscle below the lungs that helps breathing and separates the chest from the abdomen).
    • Stage IIIE: Cancer is found in lymph node groups on both sides of the diaphragm and in an area or organ other than the lymph nodes.
    • Stage IIIS: Cancer is found in lymph node groups on both sides of the diaphragm and in the spleen.
    • Stage IIIS+E: Cancer is found in lymph node groups on both sides of the diaphragm, in an area or organ other than the lymph nodes, and in the spleen.

    Stage III is also divided into stage III(1) and stage III(2) as follows:

    • Stage III(1): Cancer is found only in the upper abdomen above the renal vein.
    • Stage III(2): Cancer is found in lymph nodes in the pelvis and/or near the aorta.

    Stage IV

    In stage IV, the cancer either:

    • is found throughout one or more organs other than the lymph nodes and may be in lymph nodes near those organs; or
    • is found in one organ other than the lymph nodes and has spread to lymph nodes far away from that organ.

    Adult Hodgkin's lymphoma may be grouped for treatment as follows:

    Early Favorable

    Early favorable adult Hodgkin's lymphoma is stage I or stage II, without risk factors.

    Early Unfavorable

    Early unfavorable adult Hodgkin's lymphoma is stage I or stage II with 1 or more of the following risk factors:

    • A tumour in the chest that is larger than 1/3 of the width of the chest or at least 10 centimeters.
    • Cancer in an organ other than the lymph nodes.
    • A high sedimentation rate (in a sample of blood, the red blood cells settle to the bottom of the test tube more quickly than normal).
    • Three or more lymph nodes with cancer.
    • Symptoms such as fever, weight loss, or night sweats.

    Advanced Favorable

    Advanced favorable adult Hodgkin's lymphoma is stage III or stage IV with 3 or fewer of the following risk factors:

    • Having a low blood albumin (protein) level (below 4).
    • Having a low haemoglobin level (below 10.5).
    • Being male.
    • Being aged 45 years or older.
    • Having stage IV disease.
    • Having a high white blood cell count (15,000 or higher).
    • Having a low lymphocyte count (below 600 or less than 8% of the white blood cell count).

    Advanced Unfavorable

    Advanced unfavorable Hodgkin's lymphoma is stage III or stage IV with 4 or more of the following risk factors:

    • Having a low blood albumin (protein) level (below 4).
    • Having a low haemoglobin level (below 10.5).
    • Being male.
    • Being aged 45 years or older.
    • Having stage IV disease.
    • Having a high white blood cell count (15,000 or higher).
    • Having a low lymphocyte count (below 600 or less than 8% of the white blood cell count).

    Recurrent Adult Hodgkin's Lymphoma

    Recurrent adult Hodgkin's lymphoma is cancer that has recurred (come back) after it has been treated. The cancer may come back in the lymph system or in other parts of the body.

    Treatment Option Overview

    Key Points for This Section

    • There are different types of treatment for patients with adult Hodgkin's lymphoma.
    • Patients with Hodgkin's lymphoma should have their treatment planned by a team of doctors with expertise in treating lymphomas.
    • Three types of standard treatment are used:
    • o Chemotherapy
      o Radiation therapy
      o Surgery
    • New types of treatment are being tested in clinical trials. These include the following:
    • o High-dose chemotherapy and radiation therapy with stem cell transplant
      o Clinical trials comparing new methods of treatment

      There are different types of treatment for patients with adult Hodgkin's lymphoma.

      Different types of treatment are available for patients with adult Hodgkin's lymphoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. Before starting treatment, patients may want to think about taking part in a clinical trial. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment.

      Clinical trials are taking place in many parts of the country. Information about ongoing clinical trials is available from the NCI Web site. Choosing the most appropriate cancer treatment is a decision that ideally involves the patient, family, and health care team.

      Patients with Hodgkin's lymphoma should have their treatment planned by a team of doctors with expertise in treating lymphomas.

      Treatment will be overseen by a medical oncologist, a doctor who specializes in treating cancer. The medical oncologist may refer you to other doctors who have experience and expertise in treating adult Hodgkin's lymphoma and who specialize in certain areas of medicine. These may include the following specialists:

      • Neurosurgeon.
      • Neurologist.
      • Rehabilitation specialist.
      • Radiation oncologist.
      • Endocrinologist.
      • Haematologist.
      • Other oncology specialists.

      Three types of standard treatment are used:

      Chemotherapy

      Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the spinal column, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. Combination chemotherapy is treatment with more than one anticancer drug.

      Radiation therapy

      Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells. There are two types of radiation therapy. External radiation therapy uses a machine outside the body to send radiation toward the cancer. Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated.

      Surgery

      Laparotomy is a procedure in which an incision (cut) is made in the wall of the abdomen to check the inside of the abdomen for signs of disease. The size of the incision depends on the reason the laparotomy is being done. Sometimes organs are removed or tissue samples are taken for biopsy. If cancer is found, the tissue or organ is removed during the laparotomy.

      New types of treatment are being tested in clinical trials. These include the following:

      High-dose chemotherapy and radiation therapy with stem cell transplant

      High-dose chemotherapy and radiation therapy with stem cell transplant is a method of giving high doses of chemotherapy and radiation therapy and replacing blood-forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After therapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells.

      Clinical trials comparing new methods of treatment

      Treatment Options for Adult Hodgkin's Lymphoma

      Early Favorable Hodgkin's Lymphoma

      Treatment of early favorable Hodgkin's lymphoma may include the following:

      • Combination chemotherapy with or without radiation therapy to parts of the body with cancer.
      • Radiation therapy alone to areas of the body with cancer or to the mantle field (neck, chest, armpits).
      • Clinical trials of new combinations of chemotherapy and/or radiation therapy.

      Early Unfavorable Hodgkin's Lymphoma

      Treatment of early unfavorable Hodgkin's lymphoma may include the following:

      • Combination chemotherapy with or without radiation therapy to parts of the body with cancer.
      • Clinical trials of new combinations of chemotherapy and/or radiation therapy.

      Advanced Favorable Hodgkin's Lymphoma

      Treatment of advanced favorable Hodgkin's lymphoma may include the following:

      • Combination chemotherapy with or without radiation therapy to parts of the body with cancer.
      • Clinical trials of new combinations of chemotherapy.

      Advanced Unfavorable Hodgkin's Lymphoma

      Treatment of advanced unfavorable Hodgkin's lymphoma may include the following:

      • Combination chemotherapy.
      • Clinical trials of new combinations of chemotherapy.
      • A clinical trial of high-dose chemotherapy and stem cell transplant using the patient's own stem cells.

      Recurrent Adult Hodgkin's Lymphoma

      Treatment of recurrent Hodgkin's lymphoma may include the following:

      • Combination chemotherapy.
      • Combination chemotherapy followed by high-dose chemotherapy and stem cell transplant with or without radiation therapy.
      • Radiation therapy with or without chemotherapy.
      • Chemotherapy as palliative therapy to relieve symptoms and improve quality of life.
      • A clinical trial of high-dose chemotherapy and stem cell transplant.

HIV AND AIDS

What is HIV and AIDS?

HIV = Human Immunodeficiency Virus. HIV causes AIDS.

AIDS = Acquired Immunodeficiency Syndrome. AIDS is the final stage of the spectrum of disease caused by HIV.

AIDS (acquired immunodeficiency syndrome) was first reported in the United States in 1981 and has since become a major worldwide epidemic. AIDS is caused by HIV (human immunodeficiency virus). By killing or damaging cells of the body's immune system, HIV progressively destroys the body's ability to fight infections and certain cancers. People diagnosed with AIDS may get life-threatening diseases called opportunistic infections, which are caused by microbes such as viruses or bacteria that usually do not make healthy people sick.

More than 900,000 cases of AIDS have been reported in the United States since 1981. As many as 950,000 Americans may be infected with HIV, one-quarter of whom are unaware of their infection. The epidemic is growing most rapidly among minority populations and is a leading killer of African-American males ages 25 to 44. According to the Centers for Disease Control and Prevention (CDC), AIDS affects nearly seven times more African Americans and three times more Hispanics than whites. In recent years, an increasing number of African-American women and children are being affected by HIV/AIDS. In 2003, two-thirds of U.S. AIDS cases in both women and children were among African-Americans.

TRANSMISSION HIV is spread most commonly by having unprotected sex with an infected partner. The virus can enter the body through the lining of the vagina, vulva, penis, rectum, or mouth during sex.

Risky behaviour

HIV can infect anyone who practices risky behaviours such as

  • Sharing drug needles or syringes
  • Having sexual contact, including oral, with an infected person without using a condom
  • Having sexual contact with someone whose HIV status is unknown

Infected blood

HIV also is spread through contact with infected blood. Before donated blood was screened for evidence of HIV infection and before heat-treating techniques to destroy HIV in blood products were introduced, HIV was transmitted through transfusions of contaminated blood or blood components. Today, because of blood screening and heat treatment, the risk of getting HIV from such transfusions is extremely small.

Contaminated needles

HIV is frequently spread among injection drug users by the sharing of needles or syringes contaminated with very small quantities of blood from someone infected with the virus.

It is rare, however, for a patient to give HIV to a health care worker or vice-versa by accidental sticks with contaminated needles or other medical instruments.

Mother to child

Women can transmit HIV to their babies during pregnancy or birth. Approximately one-quarter to one-third of all untreated pregnant women infected with HIV will pass the infection to their babies. HIV also can be spread to babies through the breast milk of mothers infected with the virus. If the mother takes certain drugs during pregnancy, she can significantly reduce the chances that her baby will get infected with HIV. If health care providers treat HIV-infected pregnant women and deliver their babies by cesarean section, the chances of the baby being infected can be reduced to a rate of 1 percent. HIV infection of newborns has been almost eradicated in the United States due to appropriate treatment.

A study sponsored by the National Institute of Allergy and Infectious Diseases (NIAID) in Uganda found a highly effective and safe drug for preventing transmission of HIV from an infected mother to her newborn. Independent studies have also confirmed this finding. This regimen is more affordable and practical than any other examined to date. Results from the study show that a single oral dose of the antiretroviral drug nevirapine (NVP) given to an HIV-infected woman in labour and another to her baby within 3 days of birth reduces the transmission rate of HIV by half compared with a similar short course of AZT (Azidothymidine). For more information on preventing transmission from mother to child, go to http://aidsinfo.nih.gov/guidelines.

Saliva

Although researchers have found HIV in the saliva of infected people, there is no evidence that the virus is spread by contact with saliva. Laboratory studies reveal that saliva has natural properties that limit the power of HIV to infect, and the amount of virus in saliva appears to be very low. Research studies of people infected with HIV have found no evidence that the virus is spread to others through saliva by kissing. The lining of the mouth, however, can be infected by HIV, and instances of HIV transmission through oral intercourse have been reported.

Scientists have found no evidence that HIV is spread through sweat, tears, urine, or feces.

Casual contact

Studies of families of HIV-infected people have shown clearly that HIV is not spread through casual contact such as the sharing of food utensils, towels and bedding, swimming pools, telephones, or toilet seats.

HIV is not spread by biting insects such as mosquitoes or bedbugs.

Sexually transmitted infections

If you have a sexually transmitted infection (STI) such as syphilis, genital herpes, chlamydial infection, gonorrhea, or bacterial vaginosis appears, you may be more susceptible to getting HIV infection during sex with infected partners.

EARLY SYMPTOMS OF HIV INFECTION If you are like many people, you will not have any symptoms when you first become infected with HIV. You may, however, have a flu-like illness within a month or two after exposure to the virus. This illness may include
  • Fever
  • Headache
  • Tiredness
  • Enlarged lymph nodes (glands of the immune system easily felt in the neck and groin)

These symptoms usually disappear within a week to a month and are often mistaken for those of another viral infection. During this period, people are very infectious, and HIV is present in large quantities in genital fluids.

More persistent or severe symptoms may not appear for 10 years or more after HIV first enters the body in adults, or within 2 years in children born with HIV infection. This period of "asymptomatic" infection varies greatly in each individual. Some people may begin to have symptoms within a few months, while others may be symptom-free for more than 10 years.

Even during the asymptomatic period, the virus is actively multiplying, infecting, and killing cells of the immune system. The virus can also hide within infected cells and lay dormant. The most obvious effect of HIV infection is a decline in the number of CD4 positive T (CD4+) cells found in the blood-the immune system's key infection fighters. The virus slowly disables or destroys these cells without causing symptoms.

As the immune system worsens, a variety of complications start to take over. For many people, the first signs of infection are large lymph nodes or "swollen glands" that may be enlarged for more than 3 months. Other symptoms often experienced months to years before the onset of AIDS include

  • Lack of energy
  • Weight loss
  • Frequent fevers and sweats
  • Persistent or frequent yeast infections (oral or vaginal)
  • Persistent skin rashes or flaky skin
  • Pelvic inflammatory disease in women that does not respond to treatment
  • Short-term memory loss

Some people develop frequent and severe herpes infections that cause mouth, genital, or anal sores, or a painful nerve disease called shingles. Children may grow slowly or be sick a lot.

WHAT IS AIDS? The term AIDS applies to the most advanced stages of HIV infection. CDC developed official criteria for the definition of AIDS and is responsible for tracking the spread of AIDS in the United States.

CDC's definition of AIDS includes all HIV-infected people who have fewer than 200 CD4+ T cells per cubic millimeter of blood. (Healthy adults usually have CD4+ T-cell counts of 1,000 or more.) In addition, the definition includes 26 clinical conditions that affect people with advanced HIV disease. Most of these conditions are opportunistic infections that generally do not affect healthy people. In people with AIDS, these infections are often severe and sometimes fatal because the immune system is so ravaged by HIV that the body cannot fight off certain bacteria, viruses, fungi, parasites, and other microbes.

Symptoms of opportunistic infections common in people with AIDS include

  • Coughing and shortness of breath
  • Seizures and lack of coordination
  • Difficult or painful swallowing
  • Mental symptoms such as confusion and forgetfulness
  • Severe and persistent diarrhoea
  • Fever
  • Vision loss
  • Nausea, abdominal cramps, and vomiting
  • Weight loss and extreme fatigue
  • Severe headaches
  • Coma

Children with AIDS may get the same opportunistic infections as do adults with the disease. In addition, they also have severe forms of the typically common childhood bacterial infections, such as conjunctivitis (pink eye), ear infections, and tonsillitis.

People with AIDS are also particularly prone to developing various cancers, especially those caused by viruses such as Kaposi's sarcoma and cervical cancer, or cancers of the immune system known as lymphomas. These cancers are usually more aggressive and difficult to treat in people with AIDS. Signs of Kaposi's sarcoma in light-skinned people are round brown, reddish, or purple spots that develop in the skin or in the mouth. In dark-skinned people, the spots are more pigmented.

During the course of HIV infection, most people experience a gradual decline in the number of CD4+ T cells, although some may have abrupt and dramatic drops in their CD4+ T-cell counts. A person with CD4+ T cells above 200 may experience some of the early symptoms of HIV disease. Others may have no symptoms even though their CD4+ T-cell count is below 200.

Many people are so debilitated by the symptoms of AIDS that they cannot hold a steady job or do household chores. Other people with AIDS may experience phases of intense life-threatening illness followed by phases in which they function normally.

A small number of people first infected with HIV 10 or more years ago have not developed symptoms of AIDS. Scientists are trying to determine what factors may account for their lack of progression to AIDS, such as

  • Whether their immune systems have particular characteristics
  • Whether they were infected with a less aggressive strain of the virus
  • If their genes may protect them from the effects of HIV

Scientists hope that understanding the body's natural method of controlling infection may lead to ideas for protective HIV vaccines and use of vaccines to prevent the disease from progressing.

DIAGNOSIS Because early HIV infection often causes no symptoms, your health care provider usually can diagnose it by testing your blood for the presence of antibodies (disease-fighting proteins) to HIV. HIV antibodies generally do not reach noticeable levels in the blood for 1 to 3 months following infection. It may take the antibodies as long as 6 months to be produced in quantities large enough to show up in standard blood tests. Hence, to determine whether you have been recently infected (acute infection), your health care provider can screen you for the presence of HIV genetic material. Direct screening of HIV is extremely critical in order to prevent transmission of HIV from recently infected individuals.

If you have been exposed to the virus, you should get an HIV test as soon as you are likely to develop antibodies to the virus-within 6 weeks to 12 months after possible exposure to the virus. By getting tested early, if infected, you can discuss with your health care provider when you should start treatment to help your immune system combat HIV and help prevent the emergence of certain opportunistic infections (see section on treatment below). Early testing also alerts you to avoid high-risk behaviours that could spread the virus to others.

Most health care providers can do HIV testing and will usually offer you counselling at the same time. Of course, you can be tested anonymously at many sites if you are concerned about confidentiality.

Health care providers diagnose HIV infection by using two different types of antibody tests: ELISA and Western Blot. If you are highly likely to be infected with HIV but have been tested negative for both tests, your health care provider may request additional tests. You also may be told to repeat antibody testing at a later date, when antibodies to HIV are more likely to have developed.

Babies born to mothers infected with HIV may or may not be infected with the virus, but all carry their mothers' antibodies to HIV for several months. If these babies lack symptoms, a doctor cannot make a definitive diagnosis of HIV infection using standard antibody. Health care providers are using new technologies to detect HIV to more accurately determine HIV infection in infants between ages 3 months and 15 months. They are evaluating a number of blood tests to determine which ones are best for diagnosing HIV infection in babies younger than 3 months.

TREATMENT When AIDS first surfaced in the United States, there were no medicines to combat the underlying immune deficiency and few treatments existed for the opportunistic diseases that resulted. Researchers, however, have developed drugs to fight both HIV infection and its associated infections and cancers.

HIV infection

The Food and Drug Administration (FDA) has approved a number of drugs for treating HIV infection. The first group of drugs used to treat HIV infection, called nucleoside reverse transcriptase (RT) inhibitors, interrupts an early stage of the virus making copies of itself. These drugs may slow the spread of HIV in the body and delay the start of opportunistic infections. This class of drugs, called nucleoside analogs, include

  • AZT (Azidothymidine)
  • ddC (zalcitabine)
  • ddI (dideoxyinosine)
  • d4T (stavudine)
  • 3TC (lamivudine)
  • Abacavir (ziagen)
  • Tenofovir (viread)
  • Emtriva (emtricitabine)

Health care providers can prescribe non-nucleoside reverse transcriptase inhibitors (NNRTIs), such as

  • Delavridine (Rescriptor)
  • Nevirapine (Viramune)
  • Efravirenz (Sustiva) (in combination with other antiretroviral drugs)

FDA also has approved a second class of drugs for treating HIV infection. These drugs, called protease inhibitors, interrupt the virus from making copies of itself at a later step in its life cycle. They include

  • Ritonavir (Norvir)
  • Saquinivir (Invirase)
  • Indinavir (Crixivan)
  • Amprenivir (Agenerase)
  • Nelfinavir (Viracept)
  • Lopinavir (Kaletra)
  • Atazanavir (Reyataz)
  • Fosamprenavir (Lexiva)

FDA also has introduced a third new class of drugs, known at fusion inhibitors, to treat HIV infection. Fuzeon (enfuvirtide or T-20), the first approved fusion inhibitor, works by interfering with HIV-1's ability to enter into cells by blocking the merging of the virus with the cell membranes. This inhibition blocks HIV's ability to enter and infect the human immune cells. Fuzeon is designed for use in combination with other anti-HIV treatment. It reduces the level of HIV infection in the blood and may be active against HIV that has become resistant to current antiviral treatment schedules.

Because HIV can become resistant to any of these drugs, health care providers must use a combination treatment to effectively suppress the virus. When multiple drugs (three or more) are used in combination, it is referred to as highly active antiretroviral therapy, or HAART, and can be used by people who are newly infected with HIV as well as people with AIDS.

Researchers have credited HAART as being a major factor in significantly reducing the number of deaths from AIDS in this country. While HAART is not a cure for AIDS, it has greatly improved the health of many people with AIDS and it reduces the amount of virus circulating in the blood to nearly undetectable levels. Researchers, however, have shown that HIV remains present in hiding places, such as the lymph nodes, brain, testes, and retina of the eye, even in people who have been treated.

Side effects

Despite the beneficial effects of HAART, there are side effects associated with the use of antiviral drugs that can be severe. Some of the nucleoside RT inhibitors may cause a decrease of red or white blood cells, especially when taken in the later stages of the disease. Some may also cause inflammation of the pancreas and painful nerve damage. There have been reports of complications and other severe reactions, including death, to some of the antiretroviral nucleoside analogs when used alone or in combination. Therefore, health care experts recommend that you be routinely seen and followed by your health care provider if you are on antiretroviral therapy.

The most common side effects associated with protease inhibitors include nausea, diarrhoea, and other gastrointestinal symptoms. In addition, protease inhibitors can interact with other drugs resulting in serious side effects. Fuzeon may also cause severe allergic reactions such as pneumonia, trouble breathing, chills and fever, skin rash, blood in urine, vomiting, and low blood pressure. Local skin reactions are also possible since it is given as an injection underneath the skin.

If you are taking HIV drugs, you should contact your health care provider immediately if you have any of these symptoms.

Opportunistic infections

A number of available drugs help treat opportunistic infections. These drugs include

  • Foscarnet and ganciclovir to treat CMV (cytomegalovirus) eye infections
  • Fluconazole to treat yeast and other fungal infections
  • TMP/SMX (trimethoprim/sulfamethoxazole) or pentamidine to treat PCP (Pneumocystis carinii pneumonia)

Cancers

Health care providers use radiation, chemotherapy, or injections of alpha interferon-a genetically engineered protein that occurs naturally in the human body-to treat Kaposi's sarcoma or other cancers associated with HIV infection.

PREVENTION Because no vaccine for HIV is available, the only way to prevent infection by the virus is to avoid behaviours that put you at risk of infection, such as sharing needles and having unprotected sex.

Many people infected with HIV have no symptoms. Therefore, there is no way of knowing with certainty whether your sexual partner is infected unless he or she has repeatedly tested negative for the virus and has not engaged in any risky behaviour. You should either abstain from having sex or use male latex condoms or female polyurethane condoms, which may offer partial protection, during oral, anal, or vaginal sex. Only water-based lubricants should be used with male latex condoms.

Although some laboratory evidence shows that spermicides can kill HIV, researchers have not found that these products can prevent you from getting HIV.

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