Modern management of myelofibrosis.
The conventional treatment of myelofibrosis involves a wait-and-see approach for asymptomatic patients, oral chemotherapy for the hyperproliferative forms of the disease, androgens or erythropoietin for the anaemia, and splenectomy in selected patients. Low-dose thalidomide plus prednisone is a well-tolerated therapy for the anaemia and the thrombocytopenia of myelofibrosis, whereas imatinib has shown little efficacy. Allogeneic stem cell transplantation (allo-SCT) is the only curative therapy for myelofibrosis. Its standard modality has an associated mortality of about 30% and can be applied to younger patients with high-risk disease or resistant to conventional treatment. Reduced-intensity conditioning allo-SCT involves a low mortality and is a promising therapy for patients aged 45-70 years old with the above characteristics. Autologous SCT is a palliative therapy for patients resistant to conventional treatment who lack a suitable donor. The next candidates for the treatment of myelofibrosis are the thalidomide derivatives, the proteasome inhibitors, and vascular endothelial growth factor neutralizing antibodies.
Cervantes
Francisco
F
Haematology Department, Hospital Clínic, IDIBAPS, University of Barcelona, Barcelona, Spain. fcervan@clinic.ub.es
D000328:Adult; D020533:Angiogenesis Inhibitors / Q000627:therapeutic use; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D006801:Humans; D008875:Middle Aged; D010166:Palliative Care; D011241:Prednisone / Q000627:therapeutic use; D055728:Primary Myelofibrosis / Q000188:drug therapy / Q000601:surgery / Q000628:therapy*; D013156:Splenectomy; D033581:Stem Cell Transplantation; D013792:Thalidomide / Q000627:therapeutic use; D014182:Transplantation, Autologous; D014184:Transplantation, Homologous
D020533:Angiogenesis Inhibitors; D000893:Anti-Inflammatory Agents; D013792:Thalidomide; D011241:Prednisone