Acquired Hemophilia B Through Liver Transplantation: A Case Report and Literature Review

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.

Donor-derived disorders of hemostasis and thrombosis in liver transplantation: Considerations for deceased donor liver selection

“…In retrospect, had a history of large thigh hematomas after injury. Died from traumatic ICH Bleeding after dental procedure Prolonged aPTT corrected after transfusion of plasma; factor IX activity ~11% (mild hemophilia) Hemophilia B Jane Kurian et al [29] No genotype testing found on record Mild factor IX deficiency, with no significant history of bleeding, and no prophylactic factor replacement. Died from head trauma with massive ICH While receiving factor replacement developed femoral DVT, and subsequently perinephric hematoma on heparin drip.…”

“…However, the opposite is also true and several case reports describe the transmission of hemophilia through OLT, with phenotypes ranging from mild to severe in the recipient postoperatively (Table 3). [25][26][27][28][29][30][31][32] While numerous case reports indicate that hemophilia A donors can transmit the disorder to the transplant recipient, most recipients have mild disease due to extrahepatic synthesis of factor VIII in the recipient (Table 3).…”