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Chronic Myelogenous Leukemia

more about Chronic Myelogenous Leukemia

CML or chronic granulocytic leukemia

  • Chronic myelogenous Leukemia (CML) refers to an overproduction of white blood cells (WBC) in the bone marrow. The marrow is the site in the body where red blood cells (i.e., RBC, carry oxygen to the cells of the body), white blood cells (i.e., WBC, fight infections), and platelets (form blood clots) are formed.
  • When excess white blood cells invade the bone marrow, they also spill over into the blood. Since they consist mostly of mature granulocytes, like neutrophiles and myelocytes, they are able to function normally. As a result, symptoms are less severe compared to acute forms of Leukemia, and develop more slowly.
  • This condition has three phases, which define symptom severity:
    1. Chronic phase, in which there are only a small number of blast cells -- i.e., immature WBCs not capable of fighting infection -- in the blood or bone marrow, and thus no signs or symptoms of Leukemia.
    2. Acute or accelerated phase, with more blasts cells found -- and more symptoms.
    3. Blastic phase, with 30% of the blood cells found to be immature blast cells. Symptoms may be severe. Most cases are seen in adults, but children also develop CML.

  • Chronic phase may last for years before entering the accelerated phase.

- Chronic phase:

      1. Feeling tired
      2. Feeling discomfort in the left upper abdomen (due to the enlargement of the spleen or splenomegaly)
      3. Mild shortness of breath
      4. Low grade fever
      5. Mild paleness 
      6. Weight loss
      7. Loss of appetite
      8. Excessive sweating, especially at night
      9. Inability to tolerate warm temperature
      10. Mild bone pain
      11. Right upper abdominal fullness (enlarged liver or hepatomegaly)
  • Accelerated phase is actually a transitional phase that may last for months to years, with the above symptoms becoming more pronounced.

    1. More pronounced left upper abdominal pain (splenomegaly)
    2. Bone pain
    3. Fever
    4. Paleness
    5. Shortness of breath
    6. Lack of energy and appetite
    7. Night sweats

  • Blast crisis:

- Bleeding -- skin Bruising, petechiae (small areas of bleeding under the skin), and fever due to infections. Patients appear very sick.

  • Blast cells can also invade the brain and the skin.

  • Unknown
  • Damaged genetic material (DNA) maybe responsible

  • Often patients unknowingly in the chronic phase with mild or no complaints, will incidentally have a physical exam or a blood test that reveals splenomegaly, hepatomegaly, or increased numbers of WBC.
  • During the accelerated or blastic phases, patients may be admitted to the hospital with complaints of infection, severe anemia, or bleeding.
  • Blood tests:

    1. May show presence of (and percentage) blast cells
    2. Show increased numbers of WBC (mature granulocytes, normal lymphocytes, basophiles, and eosinophils)
    3. May show mild to moderate anemia
    4. Platelet count may vary from low to normal to high
    5. In CML, the mature cells have absent (or low) levels of leukocyte Alkaline phosphatase (an enzyme normally present in these cells).
    6. There may be an elevation of Uric acid in the blood.

  • Bone marrow content is often examined under a microscope in order to identify Leukemia type and extent of the diseases. In blast crisis and the accelerated stage, the marrow shows large numbers of WBCs (blast cells).
  • Cytogenetic testing on the cells from the bone marrow will show the Philadelphia chromosome.
  • The Polymerase Chain Reaction Test (PCR) is a sensitive and accurate way of diagnosing CML.
  • Imaging techniques of the abdomen (MRI, CAT scan, Ultrasound) show enlargement of the spleen and liver.

  • Ionizing radiation
  • Presence of Philadelphia chromosome -- an abnormal chromosome (number 22 contains the BCR gene) found in the cells of CML patients Chromosome number 9 (ABL gene) is also abnormal in CML patients. Abnormal fusion of BCR-ABL leads to formation of an abnormal protein that may be responsible for CML.
  • Benzene.

  • Myelosuppression is the main form of therapy, intended to reduce the number of white blood cells and restore the count to normal levels.
    1. Medications such as Busulfan, Hydroxyurea, Cytarabine, and Alpha Interferon are some of the medications most commonly used.
    2. Leukapheresis is a technique used to filter the blood of excess WBCs.
    3. Bone marrow transplantation is often considered for younger patients with a sibling (whose bone marrow closely matches the patient's).
  • Removal of the spleen is sometimes done if it is enlarged.

  • Contact your physician as soon as possible. Be sure to ask about any new treatments, research, clinical trials, or procedures that may be available for CML.

more about Chronic Myelogenous Leukemia

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