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CHRONIC MYELOGENOUS LEUKEMIA (CML)
  • Increased production of early progenitor stem cells and maturation arrest in the later stages of development of cells in the bone marrow
  • All bone marrow cells except the fibroblasts are involved
  • In the peripheral blood, there could be increased white cells, red cells or platelets
  • Associated with radiation exposure, viral causes found in experimental animal models
  • Philadelphia chromosome abnormality found in majority of patients. Older patients have negative Philadelphia chromosome CML
  • There are three phases spanning about 5 years
    • Chronic phase - usually responds to therapy
    • Accelerated phase
    • Blastic phase - increased amount of blasts greater than 30% - terminal phase

SIGNS AND SYMPTOMS

  • May be asymptomatic
  • Generalized weakness, secondary to anemia
  • Anemia
  • Leucocytosis
  • Bleeding
  • Infection
  • Clot formation in the legs
  • Priapism - persistent painful penile erections
  • Elevated white and red blood cells, elevated platelets, basophils, monocytes
  • Massively enlarged spleen

TREATMENT

  • Interferon (alpha): best given in the early chronic phase; induces Philadelphia chromosome suppression (cytogenetic remission)
  • Hydroxyurea to control blood counts. Dose is titrated against cell counts
  • Busulfan; Anagrelide, Thiothepa may also be used
  • Allogeneic bone marrow transplantation - best outcome obtained if done in the chronic phase
  • Leukopheresis - to be considered in pregnant women and patients who are unstable, comatose and when immediate lowering of white cells is needed to save the patient's life
  • Alpha Interferon plus low dose Cytarabine (best standard of care)
  • Patients in blastic phase are treated like acute leukemia patients
  • Donor leucocyte Infusion (DLI) should be considered in patients who relapse after Allogeneic bone marrow transplantation
  • Allogeneic stem cell transplant gives varying results but mortality rate is high.