Myelofibrosis with myeloid metaplasia

Myelofibrosis with myeloid metaplasia is a chronic myeloproliferative disorder characterized by bone marrow fibrosis and neoangiogenesis, constitutive release of a high number of CD34+ stem cells from the bone marrow, and extramedullary hematopoiesis. It presents with heterogeneous clinical features in which anemia and progression to symptomatic splenomegaly dominate. The pathogenesis is undefined. Allogeneic stem cell transplantation offers a chance of cure. Autologus stem cell transplantation and splenectomy are risky procedures that may be considered in advanced disease when conventional therapies for correcting anemia (danazol, recombinant human erythropoietin, cyclosporine) or chemotherapy for enlarging splenomegaly and myeloproliferation (hydroxy-urea or interferon alfa) have failed. Thalidomide has been tested in numerous series, and its capacity to improve anemia and thrombocytopenia while reducing splenomegaly has been documented.