A second attack of cholestasis associated with erythropoietic protoporphyria was successfully treated by plasma exchange and blood transfusion.

Abstract

Erythropoietic protoporphyria (EPP) is a rare hereditary disease that can sometimes cause acute liver failure based on cholestasis. Acute liver failure is a fatal complication and is associated with EPP in 1-4 % of patients. Although it is extremely difficult to recover from acute liver failure, we experienced an important case of EPP where the patient recovered from the first attack of cholestasis with antibiotic treatment. The patient recovered from a second attack of cholestasis with blood infusion and plasma transfusion. This case suggests that the supply of heme by blood transfusion and the elimination of excess protoporphyrin production by plasma exchange may be a useful treatment for patients with acute cholestasis associated with EPP.

• Authors: Yohji Honda, Yoshiiku Kawakami, Hiromi Kan, Hatsue Fujino, Takayuki Fukuhara, Noriaki Naeshiro, Daisuke Miyaki, Tomokazu Kawaoka, Akira Hiramatsu, Masataka Tsuge, Michio Imamura, Hideyuki Hyogo, Hiroshi Aikata, Kazuaki Chayama
• Journal: Clinical journal of gastroenterology (Clin J Gastroenterol) Vol. 7 Issue 4 Pg. 333-7 (Aug 2014) ISSN: 1865-7265 [Electronic] Japan
• PMID: 26185883 (Publication Type: Case Reports, Journal Article)

Topics

• Adult
• Cholestasis (etiology, therapy)
• Erythrocyte Transfusion
• Humans
• Male
• Plasma Exchange
• Protoporphyria, Erythropoietic (complications)
• Recurrence
• Remission Induction