RISK FACTORS: Primary MDS is associated with occupational exposure to petroleum solvents (benzene, gasoline), Secondary (therapy-related) MDS is associated with prior treatment with alkylating agents or radiation therapy
GENERAL MEASURES
β’ Immunize for pneumococcal pneumonia and influenza and hepatitis B
β’ RBC transfusions to alleviate symptoms
β’ Platelet transfusions only for bleeding or prior to surgery, in order to avoid alloimmunization
β’ Early use of antibiotics for fever, even while culture results are pending, due to quantitative and qualitative
granulocyte disorder
β’ Iron chelation therapy to avoid iron overload from chronic transfusions
DIET Reduce alcohol use. Reduce iron intake.
PATIENT EDUCATION
β’ Stop smoking
β’ Seek early medical attention for fever, bleeding, or symptoms of anemia
β’ Advise about the risks of chronic transfusion therapy
DRUG(S) OF CHOICE
β’ Only azacitidine (Vidaza) has been proven more effective for these heterogeneous disorders than supportive
care with antibiotics and transfusions as needed.
β’ azacitidine, 75 mg/m2 SC for 7 days; repeated every 28 days, decreases RBC transfusion requirements and
yields longer times to AML or death and improvements in quality of life.
β’ Intensive chemotherapy: younger patients with MDS may benefit from AML chemotherapy, especially if Auer
rods are present, but toxicity may be severe for older patients. Remission durations are variable (median, about 1 year).
β’ Allogeneic bone marrow transplantation: recommended for younger patients with HLA-matched donors to
eradicate the malignant clone and re-supply normal hematopoietic stem cells
β’ Aminocaproic acid (epsilon-aminocaproic acid, EACA) or tranexamic acid may benefit patients with chronic,
severe thrombocytopenia and bleeding.
ALTERNATIVE DRUGS
β’ Possible differentiating agents such as tretinoin (alltrans-retinoic acid); or homoharringtonine and other
hematopoietic growth factors are under investigation
β’ Danazol or prednisone may benefi t concomitant autoimmune thrombocytopenia
β’ Investigational agents: low doses of cytarabine or decitabine, 13-cis retinoic acid, interferon, cyclosporine,
granulocyte-macrophage colony stimulating factor (GMCSF) or granulocyte colony stimulating factor (G-CSF),
interleukin-3 (IL-3)
β’ Agents, such as thalidomide, that inhibit production of tumor necrosis factor in the marrow are being investigated
β’ Amifostine may stimulate proliferation of normal hematopoiesis
β’ Topotecan may have cytotoxic benefit in CMMOL
β’ Lenalidomide (Revlimid) may induce complete remissions in refractory anemia with del (5q) syndrome
β’ Vitamins, iron, corticosteroids, androgens, or thyroid hormone are rarely helpful unless evidence of a specific
deficiency exists.
PATIENT MONITORING At least monthly during supportive care. More frequently if receiving treatment.
POSSIBLE COMPLICATIONS Infection, bleeding, complications of anemia and transfusions
EXPECTED COURSE/PROGNOSIS
β’ Median survival for RA and RARS is 5 years but may extend much longer. Refractory anemia with 5q minus
syndrome is quite favorable.
β’ Median survival for RAEB, RAEBT, and CMMOL is about 1 year with half of patients evolving to AML and
the other half dying of infection or bleeding