Erythropoietic Protoporphyria-related Hepatopathy Successfully Treated with Phlebotomy

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...

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Detalles Bibliográficos
Autores principales: Yoshida, Akihiro, Hagiwara, Satoru, Watanabe, Tomohiro, Nishida, Naoshi, Ida, Hiroshi, Sakurai, Toshiharu, Komeda, Yoriaki, Yamao, Kentaro, Takenaka, Mamoru, Enoki, Eisuke, Kimura, Masatomo, Miyake, Masako, Kawada, Akira, Kudo, Masatoshi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Japanese Society of Internal Medicine 2018
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6172546/
https://www.ncbi.nlm.nih.gov/pubmed/30175727
http://dx.doi.org/10.2169/internalmedicine.0673-17
Descripción
Sumario: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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