Abstract
Hemophilia B, also called Christmas disease, is an X-linked inherited bleeding disorder that predominantly affects males, and it is caused by deficiency of coagulation factor IX. Factor IX is a coagulation factor produced in the liver. Liver transplantation from hemophilia B donors is neither widely documented nor performed due to presumed risk of developing increased bleeding tendency in the recipient due to potential acquired factor IX deficiency. In this review, we present a case of liver and kidney transplantation from a donor with mild hemophilia B to a recipient with no history of hemophilia B and literature review. This is a relatively rare situation with only two other prior case reports of acquired Hemophilia B through liver transplantation noted in 2015. The 2 cases presented in the literature lead to acquired hemophilia B in liver transplant recipients since donors were not screened due to mild disease and low suspicion level. In our case, the donor was known to have mild Hemophilia B and the recipient had a rapid decline – thus it is imperative to weigh risks and benefits of transplantation from this donor population. While those individuals with mild hemophilia B disease (and low bleeding burden) should be considered as an alternative donor for liver transplantation, appropriate counseling should be done with the recipient about possible bleeding risks and OLT should only occur after patient consent. A multidisciplinary approach should involve hematology in case factor replacement therapy is needed post transplantation and for long term follow up.
Highlights
Hemophilia B, called Christmas disease, is an X-linked inherited bleeding disorder that predominantly affects males
We present a case of liver and kidney transplantation from a donor with mild hemophilia B to a recipient with no history of hemophilia B and literature review
The donor was mildly symptomatic and not known to have a bleeding disorder as per his family members [9]. This was followed by another report in 2015 by Brunetta et al, who detailed a case of a new onset of FIX deficiency, in association with mild coagulation abnormalities following liver transplantation, attributable to an unrecognized hemophilia B in the donor
Summary
Hemophilia B, called Christmas disease, is an X-linked inherited bleeding disorder that predominantly affects males. Liver transplantation from hemophilia B donors is neither widely documented nor performed due to presumed risk of developing increased bleeding tendency in the recipient due to potential acquired factor IX deficiency. The patient was listed for liver-kidney transplantation with discussions to expand patient's pool of donors (US Centers for Disease Control and Prevention high-risk donors and hepatitis B core antibody-positive donors) due to rapidly progressive disease Prior to transplantation, he did not have any history of recurring bleeding events (no Factor IX activity level prior to pre-transplant evaluation was available, PTT was normal at 34 seconds 5 years before presentation). The patient developed Pseudomona pneumonia and developed hypoxic respiratory failure His condition continued to deteriorate and his family made the decision to withdraw care on POD 55, and replacement factor stopped. His factor IX levels eventually dropped to around 20-30% in the following days before he passed away on POD 62
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
