Prognostic factors and current practice in treatment of myelofibrosis with myeloid metaplasia: an update anno 2000.
Median survival of patients with myelofibrosis with myeloid metaplasia (MMM) ranges from 3.5 to 5 years, but there is a wide variability. The degree of anemia (Hb < 10 g/dL) is the most important prognostic factor, followed by constitutional symptoms and abnormal karyotype. In recent years, different prognostic scoring systems for MMM have been proposed. In some of them three prognostic groups (low, intermediate, and high risk) are recognized, while others recognize a high and a low-risk group only. Median survival of the low-risk group ranges from seven to nine years, while the minority of high-risk patients survive for a median of less than two years. Younger patients with MMM survive longer (median survival above ten years). Among the latter patients, based on Hb value, constitutional symptoms, and blood blast-cell percentage, two prognostic groups can also be identified, with median survival of less than three years and almost 15 years, respectively. Conventional treatment of MMM is mostly palliative and based on cytolytic treatment (usually hydroxyurea), androgen therapy and splenectomy in selected patients. Allogeneic hemopoietic transplant is a therapeutic possibility with the potential for cure in younger patients with bad prognostic features. The role in MMM of newer treatment strategies such as autologous transplantation or the administration of anti-angiogenic drugs such as thalidomide is currently being evaluated.
Cervantes
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Hematology Department, Hospital Clinic, Barcelona, Spain.
D000368:Aged; D000740:Anemia / Q000209:etiology / Q000628:therapy; D000782:Aneuploidy; D020533:Angiogenesis Inhibitors / Q000627:therapeutic use; D016026:Bone Marrow Transplantation; D019090:Case Management / Q000706:statistics & numerical data; D003131:Combined Modality Therapy; D005260:Female; D006454:Hemoglobins / Q000032:analysis; D006801:Humans; D006918:Hydroxyurea / Q000627:therapeutic use; D007621:Karyotyping; D008297:Male; D008875:Middle Aged; D010166:Palliative Care; D055728:Primary Myelofibrosis / Q000401:mortality* / Q000628:therapy; D011379:Prognosis; D011799:Quinazolines / Q000627:therapeutic use; D012306:Risk; D012720:Severity of Illness Index; D013156:Splenectomy; D016019:Survival Analysis; D013792:Thalidomide / Q000627:therapeutic use
D020533:Angiogenesis Inhibitors; D006454:Hemoglobins; D011799:Quinazolines; D013792:Thalidomide; C021139:anagrelide; D006918:Hydroxyurea