Abstract
A 58-year-old male was admitted with complaints of general fatigue and abdominal fullness on Feb. 1980. Physical examination revealed anemia and hepatosplenomegaly. Peripheral blood examination showed: RBC 341×104/cmm, Hb 9.3 g dl, Ht 30.5, WBC 12,100/cmm with leukorythroblastosis and tear drop cells and platelets 25.2×104/cmm. Bone marrow puncture, tried twice, was dry tap and a bone marrow biopsy of ileac crest showed a diffuse fibrosis and an increase of megakaryocytes. Ph1 chromosome was negative and the score of neutrophil alkaline phosphatase was 232. The diagnosis of primary myelofibrosis was made because of the lack of other causetative diseases for fibrosis. The patient was discharged on July, 1980 and followed up in the out-patient department. Two months later, a cutaneous hard nodule appeared on his forearm and disseminated to his breast and abdominal wall. Moreover, the number of peripheral white blood cell counts and the percentage of myeloblasts increased (WBC 94,800cmm, Myeloblast 32). The patient died of respiratory failure on Oct. 11, 1980. The findings of autopsy were; (1) primary myelofibrosis (2) extramedullary hematopoiesis in liver, spleen and skin, and (3) leukemic conversion. This is the second reported case of primary myelofibrosis associated with extramedullary hematopoiesis in the skin.
