Acquired Factor XI Deficiency: A Rare Complication After Liver Transplantation

Yankol, Yücel; Mecit, Nesimi; Kanmaz, Turan; Kalayoğlu, Münci

doi:10.1016/j.transproceed.2014.10.042

Cited by 7 publications

(12 citation statements)

References 10 publications

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“…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

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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

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Section: Discussionmentioning

confidence: 98%

Untitled

Milani

Aliakbarian²,

Amouian³

2018

Exp Clin Transplant

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“…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

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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.

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“…72 Liver transplantation corrects the deficiency and may prevent neurologic declines, as well. 73 Rates of intellectual disabilities ranged from 83% in a 1998 study 48 to 6% in a study of individuals identified by NBS. 68 Among 20 patients in one series, 15 survived long enough to be evaluated, and 9 of these 15 were noted to have moderate to severe intellectual disabilities.…”

Section: Argininosuccinate Lyase Deficiency/argininosuccinic Aciduriamentioning

confidence: 99%