Machado E Silva Gomide Larissa, Weihermann Viktoria, de Oliveira Isabella Corrêa, Nissel Maria Alice Zarate, Valejo Igor Raphael Mathias, da Silva Wolff Lucas, de Aguiar Alan Junior, Verona Dunia, Degraf Ygor, de Carvalho Arouca Julianna Storace, Barros Sanches João Paulo, Cabral Rodrigo Rezende Silva, Kampa Katia Cristina, de Freitas Alexandre Coutinho Teixeira, Tefili Nertan Luiz
Department of Gastrointestinal Surgery and Liver Transplantation, Clinical Hospital of the Federal University of Paraná, Curitiba, PR, Brasil.
Department of General Surgery, Clinical Hospital of the Federal University of Paraná, Curitiba, PR, Brasil.
J Surg Case Rep. 2022 Sep 5;2022(9):rjac393. doi: 10.1093/jscr/rjac393. eCollection 2022 Sep.
Hemophilia B is a recessive hereditary disease, and manifestations result from coagulation factor IX deficiency. Although improbable, as factor IX is produced exclusively in the liver, the possibility of developing the disease after transplantation represents an infrequent but potentially morbid complication. Standard laboratory tests may be insufficient to determine the probability of transmission of this pathology. This report describes the case of a patient who developed hemophilia B after liver transplantation whose donor had no prior knowledge of the disease.
血友病B是一种隐性遗传性疾病,其症状由凝血因子IX缺乏引起。虽然可能性不大,因为凝血因子IX仅在肝脏中产生,但移植后患上该疾病的可能性是一种罕见但可能致病的并发症。标准实验室检查可能不足以确定这种病理状况传播的可能性。本报告描述了一名肝移植后患上血友病B的患者的病例,其供体此前对该疾病一无所知。