General Information About Chronic Myelogenous Leukemia

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KEY POINTS

  • Chronic myelogenous leukemia is a disease in which the bone marrow makes too many white blood cells.
  • Leukemia may affect red blood cells, white blood cells, and platelets.
  • Signs and symptoms of chronic myelogenous leukemia include weight loss and tiredness.
  • Most people with CML have a gene mutation (change) called the Philadelphia chromosome.
  • Tests that examine the blood and bone marrow are used to diagnose chronic myelogenous leukemia.
  • Certain factors affect prognosis (chance of recovery) and treatment options.

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

Chronic myelogenous leukemia (also called CML or chronic granulocytic leukemia) is a slowly progressing blood and bone marrow disease that usually occurs during or after middle age, and rarely occurs in children.

ENLARGEAnatomy of the bone. The bone is made up of compact bone, spongy bone, and bone marrow. Compact bone makes up the outer layer of the bone. Spongy bone is found mostly at the ends of bones and contains red marrow. Bone marrow is found in the center of most bones and has many blood vessels. There are two types of bone marrow: red and yellow. Red marrow contains blood stem cells that can become red blood cells, white blood cells, or platelets. Yellow marrow is made mostly of fat.

Leukemia may affect red blood cells, white blood cells, and platelets.

Normally, the bone marrow makes blood stem cells (immature cells) that become mature blood cells over time. A blood stem cell may become a myeloid stem cell or a lymphoid stem cell. A lymphoid stem cell becomes a white blood cell.

A myeloid stem cell becomes one of three types of mature blood cells:

  • Red blood cellsthat carry oxygen and other substances to all tissues of the body.
  • Plateletsthat form blood clots to stop bleeding.
  • Granulocytes(white blood cells) that fight infection and disease.

ENLARGEBlood cell development. A blood stem cell goes through several steps to become a red blood cell, platelet, or white blood cell.

In CML, too many blood stem cells become a type of white blood cell called granulocytes. These granulocytes are abnormal and do not become healthy white blood cells. They are also called leukemia cells. The leukemia cells can build up in the blood and bone marrow so there is less room for healthy white blood cells, red blood cells, and platelets. When this happens, infection, anemia, or easy bleeding may occur.

Signs and symptoms of chronic myelogenous leukemia include weight loss and tiredness.

These and other signs and symptoms may be caused by CML or by other conditions. Check with your doctor if you have any of the following:

  • Feeling very tired.
  • Weight loss for no known reason.
  • Drenching 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 a protein, called tyrosine kinase, that causes too many stem cells to become white blood cells (granulocytes or blasts).

The Philadelphia chromosome is not passed from parent to child.

ENLARGEPhiladelphia chromosome. A piece of chromosome 9 and a piece of chromosome 22 break off and trade places. The BCR-ABL gene is formed on chromosome 22 where the piece of chromosome 9 attaches. The changed chromosome 22 is called the Philadelphia chromosome.

Tests that examine the blood and bone marrow are used to diagnose chronic myelogenous leukemia.

The following tests and procedures may be used:

  • Physical examand health 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) with differential: A procedure in which a sample of blood is drawn and checked for the following:
    • The number of red blood cells and platelets.
    • The number and type of white blood cells.
    • The amount of hemoglobin(the protein that carries oxygen) in the red blood cells.
    • The portion of the blood sample made up of red blood cells.

ENLARGEComplete blood count (CBC). Blood is collected by inserting a needle into a vein and allowing the blood to flow into a tube. The blood sample is sent to the laboratory and the red blood cells, white blood cells, and platelets are counted. The CBC is used to test for, diagnose, and monitor many different conditions.

  • Blood chemistry studies: A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organsand tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease.
  • Bone marrow aspiration and biopsy: The removal of bone marrow, blood, and a small piece of bone by inserting a needle into the hipbone or breastbone. A pathologistviews the bone marrow, blood, and bone under a microscope to look for abnormal cells.ENLARGEBone marrow aspiration and biopsy. After a small area of skin is numbed, a bone marrow needle is inserted into the patient’s hip bone. Samples of blood, bone, and bone marrow are removed for examination under a microscope.

One of the following tests may be done on the samples of blood or bone marrow tissue that are removed:

  • Cytogenetic analysis: A laboratory testin which the chromosomes of cells in a sample of blood or bone marrow are counted and checked for any changes, such as broken, missing, rearranged, or extra chromosomes. Changes in certain chromosomes, such as the Philadelphia chromosome, may be a sign of cancer. Cytogenetic analysis is used to help diagnose cancer, plan treatment, or find out how well treatment is working.
  • FISH (fluorescence in situ hybridization): A laboratory test used to look at and count genesor chromosomes in cells and tissues. Pieces of DNA that contain fluorescent dyes are made in the laboratory and added to a sample of a patient’s cells or tissues. When these dyed pieces of DNA attach to certain genes or areas of chromosomes in the sample, they light up when viewed under a fluorescent microscope. The FISH test is used to help diagnose cancer and help plan treatment.
  • Reverse transcription–polymerase chain reaction test (RT–PCR): A laboratory test in which the amount of a genetic substance called mRNAmade by a specific gene is measured. An enzyme called reverse transcriptase is used to convert a specific piece of RNA into a matching piece of DNA, which can be amplified (made in large numbers) by another enzyme called DNA polymerase. The amplified DNA copies help tell whether a specific mRNA is being made by a gene. RT-PCR can be used to check the activation of certain genes that may indicate the presence of cancer cells. This test may be used to look for certain changes in a gene or chromosome, which may help diagnose cancer.

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

The prognosis 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 Leukemia

KEY POINTS

  • After chronic myelogenous leukemia has been diagnosed, tests are done to find out if the cancer has spread.
  • Chronic myelogenous leukemia has 3 phases.
    • Chronic phase
    • Accelerated phase
    • Blastic phase
  • Chronic myelogenous leukemia can relapse (return) after it has been treated.

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

The extent or spread of cancer is usually described as stages. In chronic myelogenous leukemia (CML), 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 information from tests and procedures done to diagnose chronic myelogenous leukemia is also used to plan treatment.

Chronic myelogenous leukemia 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 signs or symptoms determine the phase of the disease.

Chronic phase

In chronic phase CML, fewer than 10% of the cells in the blood and bone marrow are blast cells.

Accelerated phase

In accelerated phase CML, 10% to 19% of the cells in the blood and bone marrow are blast cells.

Blastic phase

In blastic phase CML, 20% or more 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.

Chronic myelogenous leukemia can relapse (return) after it has been treated.

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

Treatment Option Overview

KEY POINTS

  • There are different types of treatment for patients with chronic myelogenous leukemia.
  • Six types of standard treatment are used:
    • Targeted therapy
    • Chemotherapy
    • Immunotherapy
    • High-dose chemotherapy with stem cell transplant
    • Donor lymphocyte infusion (DLI)
    • Surgery
  • New types of treatment are being tested in clinical trials.
  • Treatment for chronic myelogenous leukemia may cause side effects.
  • Patients may want to think about taking part in a clinical trial.
  • Patients can enter clinical trials before, during, or after starting their cancer treatment.
  • Follow-up tests may be needed.

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

Different types of treatment are available for patients with chronic myelogenous leukemia (CML). Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information about 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. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.

Six types of standard treatment are used:

Targeted therapy

Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapy may cause less harm to normal cells than chemotherapy and radiation therapy do.

  • Tyrosine kinase inhibitor therapy: This treatment blocks the enzyme, tyrosine kinase, that causes stem cellsto develop into more white blood cells (blasts) than the body needs. Imatinib mesylate (Gleevec), dasatinib, nilotinib, ponatinib, and bosutinib are tyrosine kinase inhibitors used to treat CML.

See Drugs Approved for Chronic Myelogenous Leukemia for more information.

Chemotherapy

Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them 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).

See Drugs Approved for Chronic Myelogenous Leukemia for more information.

Immunotherapy

Immunotherapy 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 biologic therapy.

  • Interferon: Interferon affects the division of cancer cells and can slow tumor

See Drugs Approved for Chronic Myelogenous Leukemia for more information.

High-dose chemotherapy with stem cell transplant

High doses of chemotherapy are given to kill cancer cells. Healthy cells, including blood-forming cells, are also destroyed by the cancer treatment. Stem cell transplant is a treatment to replace the blood-forming cells. 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 patient completes chemotherapy, 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.

See Drugs Approved for Chronic Myelogenous Leukemia for more information.

ENLARGEStem cell transplant. (Step 1): Blood is taken from a vein in the arm of the donor. The patient or another person may be the donor. The blood flows through a machine that removes the stem cells. Then the blood is returned to the donor through a vein in the other arm. (Step 2): The patient receives chemotherapy to kill blood-forming cells. The patient may receive radiation therapy (not shown). (Step 3): The patient receives stem cells through a catheter placed into a blood vessel in the chest.

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 clinical trials is available from the NCI website.

Treatment for chronic myelogenous leukemia may cause side effects.

Targeted therapy can cause side effects. The side effects you may have depend on the type of targeted therapy you receive and how your body reacts to the therapy.

The most common side effects of targeted therapy include diarrhea and liver problems. Other side effects might include problems with blood clotting and wound healing, high blood pressure, fatigue, mouth sores, nail changes, the loss of hair color, and skin problems. Skin problems might include rash or dry skin. Very rarely, a hole might form through the wall of the esophagus, stomach, small intestine, large bowel, rectum, or gallbladder.

There are medicines for many of these side effects. These medicines may prevent the side effects from happening or treat them once they occur.

Most side effects of targeted therapy go away after treatment ends.

Follow-up tests may be needed.

Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests.

Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.

Treatment of Chronic Phase Chronic Myelogenous Leukemia

For information about the treatments listed below, see the Treatment Option Overview section.

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

  • Targeted therapywith a tyrosine kinase inhibitor.
  • High-dose chemotherapywith donor stem cell transplant.
  • Chemotherapy.
  • Splenectomy.
  • A clinical trialof lower-dose chemotherapy with donor stem cell transplant.
  • A clinical trial of a new treatment.

Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.

Treatment of Accelerated Phase Chronic Myelogenous Leukemia

For information about the treatments listed below, see the Treatment Option Overview section.

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

  • Donorstem cell transplant.
  • Targeted therapywith a tyrosine kinase inhibitor (imatinib mesylate).
  • Tyrosine kinase inhibitor therapyfollowed by a donor stem cell transplant.
  • Immunotherapy(interferon) with or without chemotherapy.
  • High-dose chemotherapy.
  •  
  • Transfusiontherapy to replace red blood cells, platelets, and sometimes white blood cells, to relieve symptoms and improve quality of life.
  • A clinical trialof a new treatment.

Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.

Treatment of Blastic Phase Chronic Myelogenous Leukemia

For information about the treatments listed below, see the Treatment Option Overview section.

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

  • Targeted therapywith a tyrosine kinase inhibitor (imatinib mesylate, dasatinib, nilotinib).
  • High-dose chemotherapy.
  • Donorstem cell transplant.
  • Chemotherapyas palliative therapy to relieve symptoms and improve quality of life.
  • A clinical trialof a new treatment.

Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.

Treatment of Relapsed Chronic Myelogenous Leukemia

In relapsed CML, the number of blast cells increases after a remission. Treatment of relapsed chronic myelogenous leukemia may include the following:

  • Targeted therapywith a tyrosine kinase inhibitor (dasatinib, nilotinib, bosutinib, or higher doses of imatinib mesylate).
  • Donorstem cell transplant.
  • Chemotherapy.
  • Donor lymphocyte infusion.
  • Immunotherapy(interferon).
  • A clinical trialof new types or higher doses of targeted therapy or donor stem cell transplant.

Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.

 

For more information: https://www.cancer.gov/

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