Idiopathic myelofibrosis: historical review, diagnosis and management.
Idiopathic myelofibrosis is reviewed from several aspects. The historical development of knowledge about this disorder is discussed, from early descriptions of extramedullary hematopoiesis associated with numerous etiologies, a debate over pathogenetic mechanisms, followed by newer evidence which placed this disorder with the myeloproliferative disorders. Evidence is presented showing that idiopathic myelofibrosis is an acquired clonal disorder in terms of the hematopoietic abnormalities, but that the marrow fibrosis is a result of non-clonal disordered fibrogenesis. The clinical, laboratory and pathologic features of idiopathic myelofibrosis are discussed. The features which distinguish this disorder from the other myeloproliferative disorders, particularly chronic myelogenous leukemia are emphasized. The natural history is described together with an evaluation of accepted and experimental therapy.
Weinstein
I M
IM
Department of Medicine, UCLA School of Medicine.
D000740:Anemia / Q000209:etiology / Q000628:therapy; D001853:Bone Marrow / Q000473:pathology; D016026:Bone Marrow Transplantation; D002066:Busulfan / Q000627:therapeutic use; D003131:Combined Modality Therapy; D003937:Diagnosis, Differential; D005060:Europe; D049672:History, 19th Century; D049673:History, 20th Century; D006801:Humans; D006918:Hydroxyurea / Q000627:therapeutic use; D007155:Immunologic Factors / Q000627:therapeutic use; D055728:Primary Myelofibrosis / Q000175:diagnosis / Q000266:history / Q000473:pathology / Q000628:therapy; D011878:Radiotherapy; D013154:Spleen / Q000473:pathology; D013156:Splenectomy
D007155:Immunologic Factors; D002066:Busulfan; D006918:Hydroxyurea