続発性ヘモクロマト-シスによる心筋障害で死亡した発作性夜間血色素尿症の1例

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A 25-year-old man was admitted to our hospital because of vertigo and general fatigue in January, 1968. Laboratory findings revealed pancytopenia with relative lymphocytosis and reticulocytosis. Bone marrow aspiration showed erythroid hyperplasia. Although typical appearance of paroxysmal nocturnal hemoglobinuria (PNH) was not observed for the first several years, a Ham test in 1974 revealed positive findings, and PNH was diagnosed. In 1978, Ham, sugar-water and inulin tests' results became negative and hemoglobinuria disappeared. Hemosiderinuria, sugar-water and inulin tests were positive again in 1980, and bone marrow aspiration showed aplastic marrow. The patient died of heart failure. The total volume of blood transfusions for 12 years was approximately 8×10<SUB>4</SUB> ml. At autopsy, excessive amounts of iron deposits were found in the liver, spleen, pancreas and heart muscle. The cause of death was thought to be myocardial damage due to secondary hemochromatosis.
学校法人昭和大学・昭和医学会の論文