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


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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