Liver transplantation and haemophilia A

Lerut, Jan; Laterre, Pierre‐François; Lavenne-Pardonge, E; Donataccio, M.; Geubel, André; Reynaert, Marc; Otté, Jean-Bernard

doi:10.1016/0168-8278(95)80454-4

Cited by 22 publications

(12 citation statements)

References 16 publications

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“…Since then, multiple liver transplantations have been successful, with factor infusions provided preoperatively, perioperatively and postoperatively. Various regimes were utilized from bolus dosing to continuous infusion therapy [8,[14][15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30][31][32][33]. Factor replacement therapies achieved normal levels within 12-49 h resulting in no further need for factor replacement.…”

Section: Transplantationmentioning

confidence: 99%

Liver transplantation in the haemophilia patient

Lambing¹,

Kachalsky²,

Kuriakose³

2011

Haemophilia

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Hepatitis C is a chronic condition that many persons with haemophilia contracted in the 1980s due to the infusion of factor concentrates which did not have viral inactivation processes in place. Many patients with haemophilia are now living longer lives, well into 80 years of age, due to the improvement of their care. The effects of the HCV on the liver over time are now being realized as this population ages. Although the new treatments for hepatitis C have a prolonged response, as demonstrated by a persistent negative viral load, many haemophilia patients have either not responded to the therapy or had significant side-effects to the treatment, preventing continued therapy. Of these infected haemophiliacs with liver disease, many have demonstrated a slow progressive decline resulting in liver failure, cirrhosis or liver cancer. Liver transplant then becomes their only option. This article will review liver transplantation in the haemophilia patient highlighting three case studies demonstrating the effectiveness of specific short-term factor infusions and other haemostatic support to minimize bleeding during the surgical period. These cases confirm the opportunity for successful liver transplantation for haemophilia patients utilizing specific factor recommendations with minimal bleeding risk.

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Section: Transplantationmentioning

confidence: 99%

Liver transplantation in the haemophilia patient

Lambing¹,

Kachalsky²,

Kuriakose³

2011

Haemophilia

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“…Since then, multiple liver transplantations have been successful, with factor infusions provided preoperatively, peri-operatively and postoperatively. Various regimes were utilized from bolus dosing to continuous infusion therapy [8,9,[13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28]. Factor replacement therapies achieved normal levels within 12-49 h resulting in no further need for factor replacement.…”

Section: Review Of the Literaturementioning

confidence: 99%

Liver transplantation in the haemophilia patient

2011

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Summary. Hepatitis C is a chronic condition that many persons with haemophilia contracted in the 1980s due to the infusion of factor concentrates that did not have viral inactivation processes in place. Many patients with haemophilia are now living longer lives, well into their eighties due to the improvement of their care. The effects of the hepatitis C virus on the liver over time are now being realized as this population ages. Although the new treatments for hepatitis C have a prolonged response, as demonstrated by a persistent negative viral load, many haemophilia patients have either not responded to the therapy or had significant side effects to treatment, which prevented continued therapy. Of these infected haemophiliacs with liver disease, many have demonstrated a slow progressive decline resulting in liver failure, cirrhosis or liver cancer. Liver transplant then becomes their only option. This article will review liver transplantation in the haemophilia patient highlighting three case studies demonstrating the effectiveness of specific short-term factor infusions and other haemostatic support to minimize bleeding during the surgical period. These cases confirm the opportunity for successful liver transplantation for haemophilia patients utilizing specific factor recommendations with minimal bleeding risk.

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“…Subsequently, cure of coagulopathy after orthotopic whole liver transplantation has been reported in approximately 30 patients with hemophilia. In these recipients, liver transplantation was indicated for associated liver cirrhosis but not for cure of hemophilia 2–7. Liver transplantation is sometimes performed for other lethal metabolic liver diseases; however, the indication of liver transplantation for cure of hemophilia itself is controversial, because coagulopathy of hemophilia can be controlled by replacement with external factor VIII and the risk of whole liver transplantation is too high in this situation.…”

mentioning

confidence: 99%

Preclinical experiment of auxiliary partial orthotopic liver transplantation as a curative treatment for hemophilia

Tanaka

Kanehiro

et al. 2005

Liver Transpl

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The cause of hemophilia is deficiency of coagulation factor VIII production in the liver, which can be cured by liver transplantation. Because the hepatic function of hemophilia patients is quite normal except for production of factor VIII, auxiliary partial orthotopic liver transplantation (APOLT) is beneficial in that patient survival is secured by preserving native liver even in the event of graft loss. However, it is not known whether the graft of APOLT would be enough to cure hemophilia. We evaluated the efficacy and feasibility of APOLT for hemophilia in a canine hemophilia A model that we established. Partial left liver graft was taken from the normal donor (blood factor VIII activity > 60%). The graft was transplanted to the hemophilia beagle dog (blood factor VIII activity < 5%) after resection of the left lobe preserving native right lobe. Changes in time of blood factor VIII activity and liver function parameters were observed after APOLT. APOLT and perioperative hemostatic management were successfully performed. The blood factor VIII activity increased to 30% after APOLT, and was sustained at least 6 weeks throughout the observation period without symptoms of bleeding. The result demonstrated sustained production of factor VIII in the hemophilia recipient after APOLT. Transplantation of approximately one third of whole liver resulted in cure of hemophilia. In conclusion, it is suggested that APOLT would be feasible as a curative treatment of hemophilia A to improve quality of life of the patients. (Liver Transpl 2005;11:579-584.)

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Liver transplantation and haemophilia A

Cited by 22 publications

References 16 publications

Liver transplantation in the haemophilia patient

Liver transplantation in the haemophilia patient

Liver transplantation in the haemophilia patient

Preclinical experiment of auxiliary partial orthotopic liver transplantation as a curative treatment for hemophilia

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