Modern management of myelofibrosis

Br J Haematol. 2005 Mar;128(5):583-92. doi: 10.1111/j.1365-2141.2004.05301.x.

Abstract

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.

Publication types

  • Review

MeSH terms

  • Adult
  • Angiogenesis Inhibitors / therapeutic use
  • Anti-Inflammatory Agents / therapeutic use
  • Humans
  • Middle Aged
  • Palliative Care
  • Prednisone / therapeutic use
  • Primary Myelofibrosis / drug therapy
  • Primary Myelofibrosis / surgery
  • Primary Myelofibrosis / therapy*
  • Splenectomy
  • Stem Cell Transplantation
  • Thalidomide / therapeutic use
  • Transplantation, Autologous
  • Transplantation, Homologous

Substances

  • Angiogenesis Inhibitors
  • Anti-Inflammatory Agents
  • Thalidomide
  • Prednisone