Chronic Myelogenous Leukaemia

• Chronic myelogenous leukaemia is a disease in which the bone marrow makes too many white blood cells.
• Possible signs of chronic myelogenous leukaemia include tiredness, night sweats, and fever.
• Most people with CML have a gene mutation (change) called the Philadelphia chromosome.
• Tests that examine the blood and bone marrow are used to detect (find) and diagnose chronic myelogenous leukaemia.
• Certain factors affect prognosis (chance of recovery) and treatment options.

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

Chronic myelogenous leukaemia (also called CML or chronic granulocytic leukaemia) is a slowly progressing blood and bone marrow disease that usually occurs during or after middle age, and rarely occurs in children. Normally, the body produces bone marrow 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 CML, the body tells too many bone marrow stem cells to develop into a type of white blood cell called granulocytes. Some of these bone marrow stem cells never become mature white blood cells. These are called blasts. Over time, the granulocytes and blasts crowd out the red blood cells and platelets in the bone marrow.

This summary is about chronic myelogenous leukaemia. Refer to the following summaries for more information about leukaemia:

• Adult Acute Lymphoblastic Leukaemia Treatment
• Childhood Acute Lymphoblastic Leukaemia Treatment
• Adult Acute Myeloid Leukaemia Treatment
• Childhood Acute Myeloid Leukaemia/Other Myeloid Malignancies Treatment
• Chronic Lymphocytic Leukaemia Treatment
• Hairy Cell Leukaemia Treatment

Possible signs of chronic myelogenous leukaemia include tiredness, night sweats, and fever.

These and other symptoms may be caused by CML. Other conditions may cause the same symptoms. A doctor should be consulted if any of the following problems occur:

• Feeling very tired.
• Weight loss for no known reason.
• Night sweats.
• Fever.
• Pain or a feeling of fullness below the ribs on the left side.

Sometimes CML does not cause any symptoms at all.

Most people with CML have a gene mutation (change) called the Philadelphia chromosome.

Every cell in the body contains DNA (genetic material) that determines how the cell looks and acts. DNA is contained inside chromosomes. In CML, part of the DNA from one chromosome moves to another chromosome. This change is called the “Philadelphia chromosome.” It results in the bone marrow making an enzyme, called tyrosine kinase, that causes too many stem cells to develop into white blood cells (granulocytes or blasts).

The Philadelphia chromosome is not passed from parent to child.

Tests that examine the blood and bone marrow are used to detect (find) and diagnose chronic myelogenous leukaemia.

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 an enlarged spleen. A history of the patient’s health habits and 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.

• 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.
• 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, such as the Philadelphia chromosome.
• 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.

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 age.
• The phase of CML.
• The amount of blasts in the blood or bone marrow.
• The size of the spleen at diagnosis.
• The patient’s general health.

Stages of Chronic Myelogenous Leukaemia

Key Points for This Section

• After chronic myelogenous leukaemia has been diagnosed, tests are done to find out if the cancer has spread.
• Chronic myelogenous leukaemia has 3 phases.
o Chronic phase
o Accelerated phase
o Blastic phase

After chronic myelogenous leukaemia has been diagnosed, tests are done to find out if the cancer has spread.

Staging is the process used to find out how far the cancer has spread. There is no standard staging system for chronic myelogenous leukaemia (CML). Instead, the disease is classified by phase: chronic phase, accelerated phase, or blastic phase. It is important to know the phase in order to plan treatment. The following tests and procedures may be used to find out the phase:

• 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, such as the Philadelphia chromosome.
• 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.

Chronic myelogenous leukaemia has 3 phases.

As the amount of blast cells increases in the blood and bone marrow, there is less room for healthy white blood cells, red blood cells, and platelets. This may result in infections, anemia, and easy bleeding, as well as bone pain and pain or a feeling of fullness below the ribs on the left side. The number of blast cells in the blood and bone marrow and the severity of symptoms determine the phase of the disease.

Chronic phase

In chronic phase CML, 5% or fewer of the cells in the blood and bone marrow are blast cells.

Accelerated phase

In accelerated phase CML, 6% to 30% of the cells in the blood and bone marrow are blast cells.

Blastic phase

In blastic phase CML, more than 30% of the cells in the blood or bone marrow are blast cells. When tiredness, fever, and an enlarged spleen occur during the blastic phase, it is called blast crisis.

Relapsed Chronic Myelogenous Leukaemia

In relapsed CML, the number of blast cells increases after a remission.

Treatment Option Overview

Key Points for This Section

• There are different types of treatment for patients with chronic myelogenous leukaemia.
• Six types of standard treatment are used:
o Chemotherapy
o Other drug therapy
o Biologic therapy
o High-dose chemotherapy with stem cell transplant
o Donor lymphocyte infusion (DLI)
o Surgery

• New types of treatment are being tested in clinical trials.

There are different types of treatment for patients with chronic myelogenous leukaemia.

Different types of treatment are available for patients with chronic myelogenous leukaemia (CML). 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.

Six 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.

Other drug therapy

Imatinib (Gleevec) is a new type of cancer drug, called a tyrosine kinase inhibitor. It blocks the enzyme, tyrosine kinase, that causes stem cells to develop into more white blood cells (granulocytes or blasts) than the body needs.

Biologic therapy

Biologic therapy is a treatment that uses the patient’s immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy.

High-dose chemotherapy with stem cell transplant

High-dose chemotherapy with stem cell transplant is a method of giving high doses of 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 the patient or a donor and are frozen and stored. 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.

Donor lymphocyte infusion (DLI)

Donor lymphocyte infusion (DLI) is a cancer treatment that may be used after stem cell transplant. Lymphocytes (a type of white blood cell) from the stem cell transplant donor are removed from the donor’s blood and may be frozen for storage. The donor’s lymphocytes are thawed if they were frozen and then given to the patient through one or more infusions. The lymphocytes see the patient’s cancer cells as not belonging to the body and attack them.

Surgery

Splenectomy is surgery to remove the spleen.

New types of treatment are being tested in clinical trials.

Information about ongoing clinical trials is available from the NCI Web site.

Treatment Options for Chronic Myelogenous Leukaemia

Chronic Phase Chronic Myelogenous Leukaemia

Treatment of chronic phase chronic myelogenous leukaemia may include the following:

• High-dose chemotherapy with donor stem cell transplant.
• Biologic therapy (interferon) with or without chemotherapy.
• Other drug therapy (Gleevec).
• Chemotherapy.
• Splenectomy.
• A clinical trial of lower-dose chemotherapy with donor stem cell transplant.
• A clinical trial of a new treatment.

Information about ongoing clinical trials is available from the NCI Web site.
Accelerated Phase Chronic Myelogenous Leukaemia

Treatment of accelerated phase chronic myelogenous leukaemia may include the following:

• Stem cell transplant.
• Other drug therapy (Gleevec).
• Biologic therapy (interferon) with or without chemotherapy.
• High-dose chemotherapy.
• Chemotherapy.
• Transfusion therapy to replace red blood cells, platelets, and sometimes white blood cells, to relieve symptoms and improve quality of life.
• A clinical trial of a new treatment.

Information about ongoing clinical trials is available from the NCI Web site.
Blastic Phase Chronic Myelogenous Leukaemia

Treatment of blastic phase chronic myelogenous leukaemia may include the following:

• Other drug therapy (Gleevec).
• Chemotherapy using one or more drugs.
• High-dose chemotherapy.
• Donor stem cell transplant.
• Chemotherapy as palliative therapy to relieve symptoms and improve quality of life.
• A clinical trial of a new treatment.

Information about ongoing clinical trials is available from the NCI Web site.
Relapsed Chronic Myelogenous Leukaemia

Treatment of relapsed chronic myelogenous leukaemia may include the following:

• Donor stem cell transplant.
• Donor lymphocyte infusion.
• Biologic therapy (interferon).
• A clinical trial of biologic therapy, combination chemotherapy, or other drug therapy (Gleevec)