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Erythropoietic Protoporphyria-related Hepatopathy Successfully Treated with Phlebotomy
Akihiro Yoshida1, Satoru Hagiwara1, Tomohiro Watanabe1
1Department of Gastroenterology and Hepatology, Kindai University Faculty of Medicine, Japan.
Abstract:
A 27-year-old man bearing an erythropoietic protoporphyria (EPP)-associated ferrochelatase (FECH) mutation was admitted to our hospital for general malaise and marked elevation of the serum levels of hepatobiliary enzymes and bilirubin. Initial treatment with plasma exchange did not reduce the blood protoporphyrin or serum liver enzyme levels, so phlebotomy was started. Surprisingly, weekly phlebotomy normalized the serum levels of liver enzymes, accompanied by a marked reduction in the blood protoporphyrin levels. The clinical course of this case strongly suggests that phlebotomy may be a suitable treatment option for EPP-related hepatopathy.
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