Abstract
An autopsy case of 62-year-old male with primary myelofibrosis was reported. His course was about 13 years and leukemic transformation was seen in the last period.
At autopsy, tumor formation was revealed in the bone marrow, liver, retroperitoneal space and skin etc., in which the proliferation of myeloblasts was found as pushing through fibrous connective tissue, associated with increase of atypical megakaryocytes. Moreover, collagen fibers in the bone marrow and retroperitoneal space and so on showed fibrous long spacing-like fibers which were about 2,000Å in diameter, approximately 1,000Å periodic cross-banding.
These findings support that primary myelofibrosis should be understood as one of myeloproliferative disorders rather than as a reactive, compensatory lesion.