This paper reviews and clarifies the current understanding of the clinical and pathologic features and treatment of MF. Recent investigations indicate that MF may be mediated by platelet- and megakaryocyte-derived growth factors, impaired prostaglandin-mediated stem cell growth inhibition, or excessive endothelial cell and fibroblast proliferation. Immunologic disorders have been associated with MF. MF may be either a primary or a secondary phenomenon. Secondary MF often regresses with appropriate treatment of this underlying disorder. Primary MF may require androgen therapy, splenectomy, splenic irradiation, bone curettage, chemotherapy, or bone marrow transplantation.