症例
骨髄線維症に発作性夜間血色素尿症を合併した1例
1973 年 14 巻 7 号 p. 805-811
詳細
1973 年 14 巻 7 号 p. 805-811
A 66-year-old man with myelofibrosis and paroxysmal nocturnal hemoglobinuria was reported. The patient was diagnosed as myelofibrosis, because leukoerythroblastic anemia and hepatosplenomegaly suggesting extramedullary hematopoiesis were evident, and fibrosis of the bone marrow was confirmed by biopsy. Although he showed no hemoglobinuria since his admission, a number of signs of intravascular hemolysis such as the presence of hemosiderin in urine, the low value of haptoglobin and the increase of LDH showing isozyme pattern suggestive of hemolysis, the shortened erythrocyte life span, the positive Ham's and sugar water test, and the decrease of acetylcholineesterase activity suggested myelofibrosis complicated with paroxysmal nocturnal hemoglobinuria. He has been followed with the administration of protein anabolic hormones and washed erythrocyte transfusion.
