Chronic granulocytic leukemia

Definition

Chronic myelogenous leukemia is cancer of the bone marrow.

Alternative Names

Causes

CML can occur in adults (usually middle-aged) and children. The disease affects 1 to 2 people per 100,000 and accounts for 7 - 20% cases of leukemia. It is usually associated with a chromosome abnormality called the Philadelphia chromosome.

CML causes rapid growth of the blood-forming cells (myeloid precursors) in the bone marrow, peripheral blood, and body tissues.

Exposure to ionizing radiation is one possible trigger for this chromosome abnormality. Such exposure could occur from a nuclear disaster or from treatment of a previous cancer, like thyroid cancer or Hodgkin's lymphoma. However, the vast majority of people treated for cancer with radiation do not go on to develop leukemia. It takes many years to develop leukemia from this cause.

Symptoms

Chronic myelogenous leukemia is grouped into several phases.

The chronic phase that can last for months or years. The disease may have few or no symptoms during this time. Most people are diagnosed by during this stage, when they are being tested for something else.

The accelerated phase is a more dangerous phase, during which the leukemia cells grow more quickly. Acceleration of the disease may be associated with fever (without infection), bone pain, and a swollen spleen.

If untreated, CML progresses to the blast crisis phase. This phase is very difficult to treat and is marked by a very high count of immature white blood cells (leukemia cells). Bleeding and infection may occur due to bone marrow failure.

Other possible symptoms include:

• Fatigue
• Weakness
• Excessive sweating (night sweats)
• Low-grade fever
• Pressure under the left ribs from an swollen spleen
• Bleeding and bruising
• Sudden appearance of small red marks on the skin (petechiae)

Exams and Tests

A physical examination often reveals an enlarged spleen. A CBC shows an increased number of white blood cells.

Other tests that may be done include:

• CBC differential
• Bone marrow aspiration
• Testing for the presence of the Philadelphia chromosome
• Molecular assay for the bcr-abl gene

This disease may also alter the results of the following tests:

• Vitamin B-12 level
• Uric acid
• Platelet count

Treatment

Imatinib (Gleevec) is the first line of therapy for all patients. Gleevec blocks the Philadelphia chromosome and is assosicated with very high rates of remission. Similar drugs are being developed.

Sometimes a chemotherapy medicine called hydroxyurea (Hydrea) is used temporarily to control the white blood cell count.

The only known cure for CMS is a bone marrow transplant or stem cell transplantation.

You should discuss your options in detail with your oncologist.

Support Groups

For additional information and resources, see cancer support group and leukemia support group.

Outlook (Prognosis)

Since the introduction of Gleevec, the outlook for patients with CMS has improved dramatically. Many patients can remain in remission for many years while on this drug.

Transplantation should be considered in all patients. Long-term cure after transplantation ranges from 60 - 80%.

Possible Complications

Blast crisis can lead to complications of CML, including infection, bleeding, fatigue, unexplained fever, and kidney problems. Chemotherapy can have serious side effects, depending on the drugs used.

When to Contact a Medical Professional

Call your health care provider if you have symptoms suggestive of CML or have been diagnosed with CML and develop a fever higher than 100°F, chills, sore throat, or cough.

Prevention

Avoid exposure to radiation when possible.

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