Myelofibrosis: a review of clinical and pathologic features and treatment.

The purpose of this review is to discuss and clarify the current understanding of the pathogenesis, clinical manifestations, and treatment of MF. MF may be either a primary or secondary disorder. It is characterized by an increased deposition of bone marrow collagen, fibronectin, and laminin. Present evidence indicates that MF may be mediated by platelet or megakaryocyte growth factors, decreased prostaglandin mediated stem cell inhibition, immune complex deposition, and both fibroblast and endothelial cell proliferation. Recently acute MF has been recognized to be identical to acute megakaryocytic leukemia. Secondary MF usually responds to appropriate treatment of the underlying disease. Primary MF is usually treated by blood product support, but may be responsive to androgens, splenectomy, splenic irradiation, chemotherapy, or bone marrow ablation with marrow reconstitution.