発症後25年を経て再燃をみた発作性夜間血色素尿症の1例

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A 15-year-old adolescent was admitted to our hospital on Sept. 27, 1956, complaining of general fatigue. Physical examination revealed anemia and hemorrhagic diathesis without hepatosplenomegaly. He was diagnosed as having aplastic anemia by findings on pancytopenia, relative lymphocytosis and hypoplastic bone marrow, although hemolytic tests including Ham's test, were not performed. After discharge, he remained well until November, 1981, a month prior to his second admission. He was, by that time, 40 years old. We again noted severe anemia, icterus and hemoglobinuria. The hematocrit was 18.8%, RBC 207×10<SUP>4</SUP>/cmm, Hb 6.1g/dl. The white-cell count was 2200, with 35% neutrophils, 49% lymphocytes, 14% monocytes and 2% basophils. The platelet count was 19.5×10<SUP>4</SUP>/cmm. A bone marrow examination revealed hypercellurarity and erythroid hyperplasia. The serum LDH was 2000IU/L, total bilirubin 2.4mg/dl, and inconjugated bilirubin 1.2mg/dl. RBC survival (T1/2) was 17.5 days. Ham's test, sugar water test and Inulin test were all positive. He was diagnosed as suffering from paroxysmal nocturnal hemoglobinuria (PNH) . On first admission, marked anisocytosis and poikilocytosis were found in peripheral blood, and red urine was noted after transfusion. These findings were sometimes seen in PNH but rare in aplastic anemia, and the diagnosis in the case of the first admission should probably have been PNH. Although PNH is a chronic disease, most patients survive approximately ten years after diagnosis. This patient survived much longer, with a remission lasting 25 years.
学校法人昭和大学・昭和医学会の論文

発症後25年を経て再燃をみた発作性夜間血色素尿症の1例

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