HEMOLYTIC-ANEMIA IN PROTOPORPHYRIA - POSSIBLE PRECIPITATING ROLE OF LIVER-FAILURE AND PHOTIC STRESS

被引：26

作者：

KEY, NS ^{[1
]}

RANK, JM ^{[1
]}

FREESE, D ^{[1
]}

BLOOMER, JR ^{[1
]}

HAMMERSCHMIDT, DE ^{[1
]}

机构：

[1] UNIV MINNESOTA,SCH MED,DEPT PEDIAT,MINNEAPOLIS,MN 55455

来源：

AMERICAN JOURNAL OF HEMATOLOGY | 1992年 / 39卷 / 03期

关键词：

HEMOLYSIS; LIVER TRANSPLANTATION; PORPHYRIA;

D O I：

10.1002/ajh.2830390309

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Hemolytic anemia is not a common clinical feature in protoporphyria. In this report, we describe two patients in whom we have encountered severe hemolytic anemia. Both individuals had advanced hepatic disease as a complication of their porphyria and were undergoing orthoptic liver transplantation. The onset of hemolysis appeared to be related to the development of liver disease, and in both cases, the operative procedure acutely exacerbated the red cell destructive process. We suggest that liver involvement in protoporphyria may unmask hemolytic anemia, and that red cells in these individuals are hypersensitive to photooxidative stress, such as might occur during a prolonged operative procedure.

引用

页码：202 / 207

页数：6

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