Vnitr Lek 2020, 66(5):e43-e47 | DOI: 10.36290/vnl.2020.097

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

Pavel Polák¹, Petr Smejkal^1,2, Gabriela Romanová¹, Jiřina Zavřelová^1,2, Radomíra Hrdličková³, Šárka Blahutová³, Libuše Husová^4,5, Marta Zvarová⁶, Miroslav Penka^1,2

¹ Oddělení klinické hematologie FN Brno, pracoviště Bohunice

² Katedra laboratorních metod LF MU Brno

³ Krevní centrum FN Ostrava

⁴ Interní gastroenterologická klinika LF MU a FN Brno, pracoviště Bohunice

⁵ Centrum kardiovaskulární a transplantační chirurgie Brno

⁶ Oddělení klinické hematologie FN u sv. Anny Brno

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: hemophilia A, chronic viral hepatitis C, liver transplantation.

Published: August 1, 2020  Show citation