An Autopsy Case of Paroxysmal Nocturnal Hemoglobinuria

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  • 発作性夜間血色素尿症の 1 剖検例
  • ホッサセイ ヤカン ケッシキソ ニョウショウ ノ 1 ボウケンレイ

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Abstract

An autopsy case of Paroxysmal Nocturnal Hemogobinuria (PNH) was repoted. A 57-year-old woman was admitted to Kyorin University Hospital with complaints of general fatigue. She showed severe anemia. Ham test and Crosby test were positive and RBC acetylcholine esterase level was low. Diagnosis of PNH was made. A therapy using washed red cells transfusion, prednisolone, fluoxymesteron and ferrous sulfate were started. Fourteen month later from the diagnosis of PNH, physical examination revealed complication of femoral vein thrombosis and died of endotoxin shock due to gram negative rods. In our case, sugar water test was negative during the whole course. Thus the present case was different from the other PNH report. As PNH is rare disease, autopsy case is few. Our case was however thought to satisfy the criteria of pathological diagnosis of PNH described by Remmele and Leder.

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