Correction of factor XI deficiency by liver transplantation

Gupta, Eva; Finn, Laura S.; Johns, Gretchen J. Schaef; Pruthi, Rajiv K.; Roy, Vivek

doi:10.1097/mbc.0000000000000344

Cited by 4 publications

(5 citation statements)

References 13 publications

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“…Therapeutic agents available for management of FXI deficiency include FFP, FXI concentrate, antifibrinolytic agents such as ε‐aminocaproic acid or tranexamic acid, desmopressin, and low‐dose activated recombinant factor VII before surgery have been used to prevent bleeding complications 1 . Therapeutic plasma exchange and liver transplantation have also been reported to correct FXI deficiency 10–12 . A systematic review found that the most common treatment for patients with FXI deficiency undergoing surgery or invasive procedures was FXI concentrate (48.4%), followed by FFP (21.6%) 4 .…”

Section: Discussionmentioning

confidence: 99%

“…1 Therapeutic plasma exchange and liver transplantation have also been reported to correct FXI deficiency. [10][11][12] A systematic review found that the most common treatment for patients with FXI deficiency undergoing surgery or invasive procedures was FXI concentrate (48.4%), followed by FFP (21.6%). 4 However, the FXI preparations currently available in some European countries were not available in our hospital, and there are concerns about their potential thrombogenicity.…”

Section: Case Presentationmentioning

confidence: 99%

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Successful use of peritoneal dialysis after abdominal surgery in a patient with factor XI deficiency: A case report

Chen

Wang

et al. 2023

Seminars in Dialysis

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Factor XI (FXI) deficiency is a rare bleeding disorder of unpredictable severity that correlates poorly with FXI coagulation activity and that poses great challenges for perioperative hemostatic management and the dialysis methods potentially available to new end-stage renal disease (ESRD) patients. We describe an individual with both ESRD and severe FXI deficiency, who successfully underwent peritoneal dialysis (PD) after emergency abdominal surgery. In the traditional concept, recent abdominal surgery is a contraindication to PD, especially for patients with bleeding risk. However, this case report highlights that PD can still be an possible option for patients with FXI deficiency who have just undergone abdominal surgery; laparoscopic PD catheter placement offers a chance to establish PD access in patients traditionally viewed as noncandidates for this method. Careful perioperative management and fresh frozen plasma transfusion ensure successful surgery. This case should be of help to clinicians and patients considering PD in similar situations.

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Section: Discussionmentioning

confidence: 99%

Section: Case Presentationmentioning

confidence: 99%

Successful use of peritoneal dialysis after abdominal surgery in a patient with factor XI deficiency: A case report

Chen

Wang

et al. 2023

Seminars in Dialysis

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“…Therapeutic plasma exchange has also been reported as a means of avoiding volume overload, which can complicate traditional plasma infusion . The liver appears to be a critical site for FXI production, and FXI deficiency may be corrected by orthotopic liver transplantation …”

Section: Discussionmentioning

confidence: 99%

“…10 The liver appears to be a critical site for FXI production, and FXI deficiency may be corrected by orthotopic liver transplantation. 11,12 Unlike in Hemophilia A and B, routine prophylaxis for everyday activity is not required in individuals with FXI deficiency as they tend not to bleed spontaneously. However, at the time of major surgery or invasive procedures (eg, tonsillectomy, kidney biopsy), prophylaxis must be considered.…”

Section: Discussionmentioning

confidence: 99%

Perioperative management of a redo aortic root replacement in a patient with severe factor XI deficiency

et al. 2018

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“…Transplantace jater byla opakovaně a úspěšně použita i v léčbě jiných vrozených krvácivých stavů -např. u kongenitálního deficitu fibrinogenu, von Willebrandova faktoru, F IX či F XI (11)(12)(13)(14) Z výše uvedených prací vyplývá, že transplantace jater je u pacientů s vrozenou poruchou hemostázy možná a při důsledném vedení a monitoraci substituční terapie i relativně bezpečná, přesto je však ve střednědobém až dlouhodobém horizontu zatížena nezanedbatelnou mortalitou z jiných než hematologických příčin. Na mortalitě pacientů se mimo jiné může podílet i rekurence chronické hepatitidy ve štěpu (1).…”

Section: úVodunclassified

Liver transplantation as potential curative method in severe hemophilia A: case report and literature review

Polák¹,

Šmejkal²,

Romanová³

et al. 2020

Vnitř Lék

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Oddělení klinické hematologie FN Brno, pracoviště Bohunice 2 Katedra laboratorních metod LF MU Brno 3 Krevní centrum FN Ostrava 4 Interní gastroenterologická klinika LF MU a FN Brno, pracoviště Bohunice 5 Centrum kardiovaskulární a transplantační chirurgie Brno 6 Oddělení klinické hematologie FN u sv. Anny Brno Autoři popisují případ ortotopické transplantace jater pro cirhózu v důsledku chronické virové hepatitidy C u pacienta s těžkou hemofilií A. Předoperační farmakokinetická studie s rekombinantním faktorem VIII (F VIII) potvrdila uspokojivé in vivo recovery 2,1 %. Perioperačně byl podán bolus F VIII v dávce 52 j/kg hmotnosti s dosažením požadované aktivity F VIII nad 100,0 %. Celkem bylo perioperačně podáno 30 000 j rekombinantního F VIII, 3 trombocytární koncentráty, 2 erytrocytární koncentráty, 5 transfuzních jednotek (TU) protivirově ošetřené plazmy, jedna TU čerstvě zmražené plazmy a 3 500 j antitrombinu. Perioperačně ani pooperačně nebyly zaznamenány krvácivé komplikace, substituce F VIII byla ukončena 3. pooperační den. Pacient byl propuštěn do domácího ošetřování 20. den po transplantaci.Klíčová slova: hemofilie A, chronická virová hepatitida C, transplantace jater.Liver transplantation as potential curative method in severe hemophilia A: case report and literature reviewThe authors present clinical case of orthotopic liver transplantation for cirhosis due to chronic viral hepatitis C in a subject with severe hemophilia A. Preoperatively performed pharmacokinetic study with recombinant F VIII confirmed satisfactory in vivo recovery of 2.1 %. A bolus application of 52 units F VIII/kg body weight with target F VIII activity over 100.0 % was administred shortly before the transplantation started. Totally, 30 000 units of recombinant F VIII, 3 thrombocyte concentrates, 2 erythrocyte concentrates, 5 units of virally inactivated plasma, 1 unit of fresh frozen plasma and 3 500 antithrombin units were used. There were no perioperative or postoperative bleeding complications, F VIII substitution was stopped on postoperative day 3. The patient was discharged on twentieth postoperative day.

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Correction of factor XI deficiency by liver transplantation

Cited by 4 publications

References 13 publications

Successful use of peritoneal dialysis after abdominal surgery in a patient with factor XI deficiency: A case report

Successful use of peritoneal dialysis after abdominal surgery in a patient with factor XI deficiency: A case report

Perioperative management of a redo aortic root replacement in a patient with severe factor XI deficiency

Liver transplantation as potential curative method in severe hemophilia A: case report and literature review

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