Sickle-Cell Disease: The Aplastic Crisis and Erythroid Maturation Defect Occurring Simultaneously in Three Members of a Family

A.M.A. archives of internal medicine Pub Date : 1960-01-01 DOI:10.1001/ARCHINTE.1960.00270130092011

G. Hilkovitz

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引用次数: 10

Abstract

It is now recognized that bone-marrow failure rather than hyperhemolysis underlies the crises in sickle-cell disease.1,2Bonemarrow failure has been reported in Cooley's hemolytic anemia,3hereditary spherocytosis,4and paroxysmal nocturnal hemoglobinuria.5Maturation arrest of the neutrophil polymorphonuclear leukocytes has been noted in sickle-cell disease.6Infection has been thought to precipitate bone-marrow aplasia in sickle-cell disease1,2and in hereditary spherocytosis.7The occurrence of "aplastic crisis" in several members of a family has been recorded in hereditary spherocytosis7and in sickle-cell disease.17Erythroblastopenia has been noted during the course of viral pneumonia in patients without hemolytic blood diseases,8and the same communication records eosinophilia in patients who had erythrocytic aplasia after exposure to allergens. The association of chronic hemolytic anemias with megaloblastic arrest of the bone marrow has been documented. Some of these responded to vitamin B12therapy,9some were associated with pregnancy,10and in others dietary deficiency of folie acid

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镰状细胞病:在一个家庭的三个成员中同时发生的再生危象和红细胞成熟缺陷

现在人们认识到，镰状细胞病的危险是骨髓衰竭而不是高溶血。骨髓功能衰竭在Cooley's溶血性贫血，3遗传性球形红细胞增多症，4和阵发性夜间血红蛋白尿中都有报道。中性粒细胞多形核白细胞成熟阻滞已在镰状细胞病中被注意到。感染被认为是镰状细胞病和遗传性球形细胞增多症患者骨髓发育不全的原因。在遗传性球形红细胞增多症和镰状细胞病中，一个家族中几个成员出现“再生危机”的记录是有的。17在没有溶血性血液病的病毒性肺炎患者中发现了红细胞减少，8在接触过敏原后出现红细胞发育不全的患者中也发现了嗜酸性粒细胞增多。慢性溶血性贫血与骨髓巨幼细胞骤停的关系已被证实。其中一些人对维生素b12治疗有反应，一些人与怀孕有关，10还有一些人缺乏叶酸

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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A.M.A. archives of internal medicine

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