抄録
A case of primary myelofibrosis was reported. A 52-year-old man was admitted to the Niigata University Hospital because of abdominal swelling and pain in December 1968. He had leuco-erythroblastic anemia and marked hepatosplenomegaly. Bone marrow puncture yielded no aspirate. Ferrokinetic study showed ineffective erythropoiesis and extramedullary hematopoiesis. Bone marrow biopsy revealed fibrosis. Splenectomy was performed in March 1971. The spleen weighed 4,000 gm. and measured 30×19.5×12 cm. Extramedullary hematopoiesis was observed in the spleen, the liver and the lymph nodes. After the splenectomy severe anemia and marked increase of both platelets and nucleated cells in the peripheral blood were evident. In March 1972, huge subcutaneous hematoma in the back appeared following strenuous muscle movements, and then hemolytic reaction occured after incompatible blood transfusions. Irregular antibodies including anti-E, anti-c and anti-Jkb antibodies were detected by serological examinations.
On the other hand, deformed spherocytes and spheric erythroblasts with a few hollows on the surface were observed by scanning electron microscopy. Suddenly, in November 1974, the patient was complicated by transverse myelopathy (Xth thoracic segment).
But he has survived for 52 months since the splenectomy.
