Chronic myelogenous leukemia (CML)

Answer 1

Definition

Chronic myelogenous leukemia is cancer that starts inside bone marrow, the soft tissue inside bones that helps form blood cells. The cancer grows from cells that produce white blood cells.

See also:

• Acute myeloid leukemia (AML)
• Chronic lymphocytic leukemia (CLL)
• Leukemia

Alternative Names

CML; Chronic granulocytic leukemia; Leukemia - chronic granulocytic (CML)

Causes, incidence, and risk factors

CML most often occurs in middle-aged adults and in children. The disease affects 1 to 2 people per 100,000 and makes up 7 - 20% cases of leukemia.

It is usually associated with a chromosome abnormality called the Philadelphia chromosome.

Radiation increases the risk of developing CML. Exposure may occur from:

• High-dose radiation treatments used in the past to treat thyroid cancer or Hodgkin's lymphoma
• Nuclear disaster

It takes many years to develop leukemia from this cause. However, most people treated for cancer with radiation do not go on to develop leukemia, and most patients with CML have not been exposed to radiation.

Symptoms

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

Chronic myelogenous leukemia is grouped into several phases:

• Chronic
• Accelerated
• Blast crisis

The chronic phase can last for months or years. The disease may have few or no symptoms during this time. Most people are diagnosed during this stage, when they are having blood tests done for other reasons.

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

If untreated, CML progresses to the blast crisis phase. Bleeding and infection may occur due to bone marrow failure. Other possible symptoms include:

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

Signs and tests

A physical examination often reveals a swollen spleen. A complete blood count (CBC) shows an increased number of white blood cells.

Other tests that may be done include:

• Bone marrow aspiration and biopsy
• CBC with white blood cell differential
• Blood and bone marrow testing for the presence of the Philadelphia chromosome

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

• Platelet count
• Uric acid

Treatment

Imatinib (Gleevec) is the first-line therapy for everyone with CML. Gleevec is a pill, taken by mouth. It is associated with very high rates of remission and survival. New medications similiar to Gleevec include dasatinib (Sprycel) and nilotinib (Tasigna).

Sometimes a chemotherapy medicine called hydroxyurea (Hydrea) is used temporarily to reduce the white blood cell count if it is very high at diagnosis.

The blast crisis phase is very difficult to treat, because it is marked by a very high count of immature white blood cells (leukemia cells). It is treated similarly to acute myeloid leukemia (AML) or acute lymphoid leukemia (ALL).

The only known cure for CML is a bone marrow transplant or stem cell transplant. You should discuss your options in detail with your oncologist.

Support Groups

See:

• Cancer support group
• Leukemia support group

Expectations (prognosis)

Since the introduction of Gleevec, the outlook for patients with CML has improved dramatically. When the signs and symptoms of CML go away, you are said to be in remission. Many patients can remain in remission for many years while on this drug.

Stem cell tansplantation should be considered in patients whose disease comes back after initial treatment with imatinib (Gleevec). Long-term cure after transplantation ranges from 60 - 80%.

Complications

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

Calling your health care provider

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

Prevention

Avoid exposure to radiation when possible.

References

Abeloff MD, Armitage JO, Niederhuber JE, Kastan MB, McKenna WG, eds. Abeloff's Clinical Oncology. 4th ed. Philadelphia, Pa: Elsevier Churchill Livingstone; 2008:chap 107.

Answer 2

Definition

Chronic myelogenous leukemia is cancer that starts inside bone marrow, the soft tissue inside bones that helps form blood cells. The cancer grows from cells that produce white blood cells.

See also:

• Acute myeloid leukemia (AML)
• Chronic lymphocytic leukemia (CLL)
• Leukemia

Alternative Names

CML; Chronic granulocytic leukemia; Leukemia - chronic granulocytic

Causes, incidence, and risk factors

CML most often occurs in middle-aged adults and in children.

It is usually associated with a chromosome abnormality called the Philadelphia chromosome.

Radiation exposure can increase your risk of developing CML. You may be exposed to radiation from:

• High-dose radiation treatments used in the past to treat thyroid cancer or Hodgkin's lymphoma
• Nuclear disaster

It takes many years to develop leukemia from this cause. However, most people treated for cancer with radiation do not go on to develop leukemia, and most patients with CML have not been exposed to radiation.

Symptoms

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

Chronic myelogenous leukemia is grouped into several phases:

• Chronic
• Accelerated
• Blast crisis

The chronic phase can last for months or years. The disease may have few or no symptoms during this time. Most people are diagnosed during this stage, when they are having blood tests done for other reasons.

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

If untreated, CML progresses to the blast crisis phase. Bleeding and infection may occur due to bone marrow failure. Other possible symptoms include:

• Bleeding and bruising
• Excessive sweating (night sweats)
• Fatigue
• Low-grade fever
• Pressure under the lower left ribs from a swollen spleen
• Rash - small pinpoint red marks on the skin (petechiae)
• Weakness

Signs and tests

A physical examination often reveals a swollen spleen. A complete blood count (CBC) shows an increased number of white blood cells.

Other tests include:

• Bone marrowbiopsy
• Blood and bone marrow testing for the presence of the Philadelphia chromosome
• Platelet count

Treatment

A medicine called Imatinib (Gleevec) is the first treatment for everyone with CML. Gleevec is a pill, taken by mouth. It is associated with very high rates of remission and survival. New medications similiar to Gleevec include dasatinib (Sprycel) and nilotinib (Tasigna).

Sometimes a chemotherapy medicine called hydroxyurea (Hydrea) is used temporarily to reduce the white blood cell count if it is very high at diagnosis.

The blast crisis phase is very difficult to treat, because it is marked by a very high count of immature white blood cells (leukemia cells). It is treated similarly to acute myeloid leukemia (AML) or acute lymphoid leukemia (ALL).

The only known cure for CML is a bone marrow transplant or stem cell transplant. You should discuss your options in detail with your oncologist.

Support Groups

See:

• Cancer support group
• Leukemia support group

Expectations (prognosis)

Since the introduction of Gleevec, the outlook for patients with CML has improved dramatically. When the signs and symptoms of CML go away, you are said to be in remission. Many patients can remain in remission for many years while on this drug.

Stem cell tansplantation is usually considered in patients whose disease comes back after taking imatinib (Gleevec).

Complications

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

Calling your health care provider

Call your health care provider if you have symptoms 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.

References

Kantarjian H, O'Brien S. The chronic leukemias. In: Goldman L, Ausiello D, eds. Cecil Textbook of Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007: chap 195.

National Comprehensive Cancer Network. National Comprehensive Cancer Network Clinical Practice Guidelines in Oncology: Chronic Myelogenous Leukemia. 2011. Version 2.2011.

Reviewed By

Review Date: 02/28/2011

David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.