Hemolytic anemia in protoporphyria: Possible precipitating role of liver failure and photic stress

Nigel S. Key, Jeffrey M. Rank, Deborah Freese, Joseph R. Bloomer, Dale E Hammerschmidt

Research output: Contribution to journalArticlepeer-review

29 Scopus citations

Abstract

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.

Original languageEnglish (US)
Pages (from-to)202-207
Number of pages6
JournalAmerican Journal of Hematology
Volume39
Issue number3
DOIs
StatePublished - Mar 1992

Keywords

  • hemolysis
  • liver transplantation
  • porphyria

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