HODGKIN'S DISEASE (Hodgkin'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.