Hemophilia B acquired through liver transplantation

Brunetta, Denise Menezes; Carneiro-Silva, Fabiana Aguiar; Vasconcelos, J.B.M.; Ribeiro, Rosângela de Albuquerque; Mesquita, D.F.G.; Coelho, Gustavo; Barroso-Duarte, Fernando; Garcia, José Huygens Parente

doi:10.1002/lt.24364

Cited by 6 publications

(10 citation statements)

References 5 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…This is a relatively rare situation with only two other prior case reports of acquired Hemophilia B through liver transplantation noted in 2015 [9,10]. The first report in literature was in 2015 by Bergstrom and colleagues who described an OLT in a 19 months old boy due to biliary atresia.…”

Section: Discussionmentioning

confidence: 95%

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

Kurian¹,

Drelich²,

Rizk³

2020

AJIM

View full text Add to dashboard Cite

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.

show abstract

Section: Discussionmentioning

confidence: 95%

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

Kurian¹,

Drelich²,

Rizk³

2020

AJIM

View full text Add to dashboard Cite

show abstract

“…Our review of the literature [6][7][8][9] revealed that transmission of blood coagulation disorder from liver donor to recipient could lead to brain hemorrhage and brain death in donors. There is no specific screening procedure recommended for detection of these rare inherited genetic disorders in deceased donors with the absence of medical or family history.…”

Section: Discussionmentioning

confidence: 98%

Untitled

Milani

Aliakbarian²,

Amouian³

2018

Exp Clin Transplant

View full text Add to dashboard Cite

Liver transplant is a life-saving procedure in patients with end-stage liver disease. However, this procedure may be associated with transmission of various deficiencies of proteins synthesized by the liver. Factor I (fibrinogen) deficiency is one of the rare inherited coagulation disorders with an extremely low risk of transmission by liver transplant. We report a case of a patient with no inherited coagulation disorders but who demonstrated disturbance of fibrinogen after liver transplant. This case highlights the ever-present risk of donor-to-recipient disease transmission during transplant and emphasizes the difficulty in procuring organs from donors in which standard blood tests are insufficient to determine the likelihood of this event. Key words: Blood coagulation disorders, Fibrinogen, Inherited transmission IntroductionThe liver donor was a 34-year-old nullipara woman who was declared brain dead after spontaneous intracranial hemorrhage. Her past medical history was mainly unknown. The donor's laboratory tests on admission showed prothrombin time of 17.3 seconds (normal range, 11-13 s), internationalized normalized ratio (INR) of 1.62, activated partial thromboplastin time of 32 seconds, and a normal platelet count, hemoglobin, and other routine tests. Ultrasonographic examination of the donor's abdomen before organ procurement showed liver having normal parenchymal echogenicity without pathologic lesions. In addition, the donor's pretransplant biopsy did not reveal any pathologic findings, such as marked steatohepatitis, fibrosis, necrosis, or malignancy. Figure 1 shows histology of the liver taken by needle biopsy on the day of organ procurement.The recipient was a 56-year-old man with endstage liver disease secondary to hepatitis B. Model for End-Stage Liver Disease score was 29. Neither he nor his family reported a history of bleeding disorders. The patient underwent deceased-donor liver transplant in November 2014. Duration of the procedure was 6 hours, with total cold ischemic time of 189 minutes and total warm ischemic time of 52 minutes. There was no unusual bleeding during the operation. The total blood loss was 3.6 L. The patient

show abstract

“…8 Previous reports have documented donor-to-recipient transmission of protein C deficiency with dysfibrinogenemia, protein S, factor I (fibrinogen), factor VII, factor VIII, and factor XI deficiencies. [9][10][11][12][13][14][15][16] Indeed, OLT may correct hepatic coagulation abnormalities, including protein C, protein S, 10 hemophilia, 17,18,19 and antithrombin III deficiencies. In contrast, transplantation of livers with a synthetic defect of a particular factor such as protein C, protein S, or antithrombin III deficiency may impose a coagulation abnormality upon these recipients that is not benign ( Table 2).…”

Section: Discussionmentioning

confidence: 99%

Donor-to-recipient transmission of factor XII deficiency by orthotopic liver transplantation

Elsiesy

Shawakat

Al–Hamoudi

et al. 2019

Baylor University Medical Center Proceedings

View full text Add to dashboard Cite

Transmission of congenital clotting factor deficiencies following orthotopic liver transplantation is rare. There has been one reported case of donor-to-recipient transmission of factor XII deficiency in a transplant, and we report the second case. KEYWORDS Coagulation; factor XII deficiency; liver transplantation; primary hyperoxaluria O rthotopic liver transplantation (OLT) may be associated with donor-transmitted deficiencies of coagulation factors due to lack of proteins synthesized by the liver. Some of these deficiencies do not have clinical significance, whereas others can lead to bleeding or thrombotic complications after OLT.

show abstract

Hemophilia B acquired through liver transplantation

Cited by 6 publications

References 5 publications

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

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

Untitled

Donor-to-recipient transmission of factor XII deficiency by orthotopic liver transplantation

Contact Info

Product

Resources

About