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Hemolytic anemia in protoporphyria: Possible precipitating role of liver failure and photic stress
Author(s) -
Key Nigel S.,
Rank Jeffrey M.,
Freese Deborah,
Bloomer Joseph R.,
Hammerschmidt Dale E.
Publication year - 1992
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.2830390309
Subject(s) - erythropoietic protoporphyria , medicine , hemolytic anemia , hemolysis , anemia , complication , porphyria , liver transplantation , disease , protoporphyrin , liver disease , immunology , gastroenterology , transplantation , biology , biochemistry , porphyrin
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.