Liver transplantation as potential curative method in severe hemophilia A: case report and literature review

Czech version

Authors: Pavel Polák ¹; Petr Smejkal ^1,2; Gabriela Romanová ¹; Jiřina Zavřelová ^1,2; Radomíra Hrdličková ³; Šárka Blahutová ³; Libuše Husová ^4,5; Marta Zvarová ⁶; Miroslav Penka ^1,2
Authors‘ workplace: Oddělení klinické hematologie FN Brno, pracoviště Bohunice ¹; Katedra laboratorních metod LF MU Brno ²; Krevní centrum FN Ostrava ³; Interní gastroenterologická klinika LF MU a FN Brno, pracoviště Bohunice ⁴; Centrum kardiovaskulární a transplantační chirurgie Brno ⁵; Oddělení klinické hematologie FN u sv. Anny Brno ⁶
Published in: Vnitř Lék 2020; 66(5): 85-89
Category: Case Report

Overview

The authors present clinical case of orthotopic liver transplantation for cirhosis due to chronic viral hepatitis C in a subject with severe hemophilia A. Preoperatively performed pharmacokinetic study with recombinant F VIII confirmed satisfactory in vivo recovery of 2.1 %. A bolus application of 52 units F VIII/kg body weight with target F VIII activity over 100.0 % was administred shortly before the transplantation started. Totally, 30 000 units of recombinant F VIII, 3 thrombocyte concentrates, 2 erythrocyte concentrates, 5 units of virally inactivated plasma, 1 unit of fresh frozen plasma and 3 500 antithrombin units were used. There were no perioperative or postoperative bleeding complications, F VIII substitution was stopped on postoperative day 3. The patient was discharged on twentieth postoperative day.

Keywords:

liver transplantation – hemophilia A – chronic viral hepatitis C

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