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. 1996 Dec;39(6):870–875. doi: 10.1136/gut.39.6.870

Liver transplantation for haemophiliacs with hepatitis C cirrhosis.

M McCarthy 1, E Gane 1, S Pereira 1, C J Tibbs 1, N Heaton 1, M Rela 1, H Hambley 1, R Williams 1
PMCID: PMC1383463  PMID: 9038673

Abstract

BACKGROUND: Experience of liver transplantation in haemophiliacs with end stage hepatitis C liver disease is limited and particularly difficult questions are raised when there is also HIV infection. AIMS: This is the first report in Great Britain to describe the operative replacement therapy and initial outcome in four haemophiliacs with end stage HCV cirrhosis. PATIENTS: Two patients had factor VIII, one had factor IX, and one had factor X deficiency. One patient had also contracted HIV infection from factor replacement but had no AIDS defining illnesses. METHODS: Intraoperatively patients were given either factor VIII infusions, factor IX bolus, or fresh frozen plasma, according to formulae devised to calculate exact clotting factor requirements. Baseline preoperative coagulation studies included prothrombin time, activated partial thromboplastin time, fibrinogen concentrations, and factor VIII, IX, and X concentrations. Factor concentrations were then assayed at 12, 24, 48, and 72 hours postoperatively. RESULTS: Postoperatively all patients had coagulation factor concentrations sustained within the normal ranges by 72 hours unsupported (137, 125, 95, 104 IU/dl), representing de novo synthesis by the graft. Transfusion requirements during the operative and immediate post-transplant period were no greater than those of patients without clotting disorders. Two patients had episodes of bleeding postoperatively, one of which was fatal, occurring at the site of a previous untreated subdural bleed. In both instances the bleeding occurred in the presence of normal concentrations of clotting factor. The remaining three patients are at 6, 6, and 12 months post-transplant and remarkably improved clinically with sustained factor concentrations. One patient has evidence of graft dysfunction from HCV recurrence and all have evidence of recurrent viraemia with HCV on polymerase chain reaction studies. CONCLUSIONS: Orthotopic liver transplantation should be considered in haemophiliac patients with end stage liver disease from hepatitis C infection with or without concomitant HIV infection. Their clinical condition is likely to be greatly improved by orthotopic liver transplantation and the haemophilia cured with only a small risk of severe graft dysfunction from recurrent HCV infection.

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Selected References

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  1. Bontempo F. A., Lewis J. H., Gorenc T. J., Spero J. A., Ragni M. V., Scott J. P., Starzl T. E. Liver transplantation in hemophilia A. Blood. 1987 Jun;69(6):1721–1724. [PMC free article] [PubMed] [Google Scholar]
  2. Castro A., Ros J., Jiménez W., Clària J., Llibre J., Leivas A., Arroyo V., Rivera F., Rodés J. Intracellular calcium concentration in vascular smooth muscle cells of rats with cirrhosis. J Hepatol. 1994 Oct;21(4):521–526. doi: 10.1016/s0168-8278(94)80096-0. [DOI] [PubMed] [Google Scholar]
  3. Eyster M. E., Diamondstone L. S., Lien J. M., Ehmann W. C., Quan S., Goedert J. J. Natural history of hepatitis C virus infection in multitransfused hemophiliacs: effect of coinfection with human immunodeficiency virus. The Multicenter Hemophilia Cohort Study. J Acquir Immune Defic Syndr. 1993 Jun;6(6):602–610. [PubMed] [Google Scholar]
  4. Eyster M. E., Fried M. W., Di Bisceglie A. M., Goedert J. J. Increasing hepatitis C virus RNA levels in hemophiliacs: relationship to human immunodeficiency virus infection and liver disease. Multicenter Hemophilia Cohort Study. Blood. 1994 Aug 15;84(4):1020–1023. [PubMed] [Google Scholar]
  5. Féray C., Gigou M., Samuel D., Paradis V., Wilber J., David M. F., Urdea M., Reynes M., Bréchot C., Bismuth H. The course of hepatitis C virus infection after liver transplantation. Hepatology. 1994 Nov;20(5):1137–1143. doi: 10.1002/hep.1840200506. [DOI] [PubMed] [Google Scholar]
  6. Gibas A., Dienstag J. L., Schafer A. I., Delmonico F., Bynum T. E., Schooley R., Rubin R. H., Cosimi A. B. Cure of hemophilia A by orthotopic liver transplantation. Gastroenterology. 1988 Jul;95(1):192–194. doi: 10.1016/0016-5085(88)90310-1. [DOI] [PubMed] [Google Scholar]
  7. König V., Bauditz J., Lobeck H., Lüsebrink R., Neuhaus P., Blumhardt G., Bechstein W. O., Neuhaus R., Steffen R., Hopf U. Hepatitis C virus reinfection in allografts after orthotopic liver transplantation. Hepatology. 1992 Nov;16(5):1137–1143. doi: 10.1002/hep.1840160506. [DOI] [PubMed] [Google Scholar]
  8. Lerut J. P., Laterre P. F., Lavenne-Pardonge E., Donataccio M., Geubel A., Reynaert M. S., Otte J. B. Liver transplantation and haemophilia A. J Hepatol. 1995 May;22(5):583–585. doi: 10.1016/0168-8278(95)80454-4. [DOI] [PubMed] [Google Scholar]
  9. Lewis J. H., Bontempo F. A., Spero J. A., Ragni M. V., Starzl T. E. Liver transplantation in a hemophiliac. N Engl J Med. 1985 May 2;312(18):1189–1190. doi: 10.1056/NEJM198505023121812. [DOI] [PMC free article] [PubMed] [Google Scholar]
  10. Merion R. M., Delius R. E., Campbell D. A., Jr, Turcotte J. G. Orthotopic liver transplantation totally corrects factor IX deficiency in hemophilia B. Surgery. 1988 Nov;104(5):929–931. [PubMed] [Google Scholar]
  11. Phillips A. N., Sabin C. A., Elford J., Bofill M., Janossy G., Lee C. A. Use of CD4 lymphocyte count to predict long-term survival free of AIDS after HIV infection. BMJ. 1994 Jul 30;309(6950):309–313. doi: 10.1136/bmj.309.6950.309. [DOI] [PMC free article] [PubMed] [Google Scholar]
  12. Sawada M., Suzumura A., Marunouchi T. Down regulation of CD4 expression in cultured microglia by immunosuppressants and lipopolysaccharide. Biochem Biophys Res Commun. 1992 Dec 15;189(2):869–876. doi: 10.1016/0006-291x(92)92284-5. [DOI] [PubMed] [Google Scholar]
  13. Schwarz A., Offermann G., Keller F., Bennhold I., L'age-Stehr J., Krause P. H., Mihatsch M. J. The effect of cyclosporine on the progression of human immunodeficiency virus type 1 infection transmitted by transplantation--data on four cases and review of the literature. Transplantation. 1993 Jan;55(1):95–103. doi: 10.1097/00007890-199301000-00019. [DOI] [PubMed] [Google Scholar]
  14. Telfer P., Sabin C., Devereux H., Scott F., Dusheiko G., Lee C. The progression of HCV-associated liver disease in a cohort of haemophilic patients. Br J Haematol. 1994 Jul;87(3):555–561. doi: 10.1111/j.1365-2141.1994.tb08312.x. [DOI] [PubMed] [Google Scholar]
  15. Tzakis A. G., Cooper M. H., Dummer J. S., Ragni M., Ward J. W., Starzl T. E. Transplantation in HIV+ patients. Transplantation. 1990 Feb;49(2):354–358. doi: 10.1097/00007890-199002000-00025. [DOI] [PMC free article] [PubMed] [Google Scholar]
  16. Webster W. P., Mandel S. R., Reddick R. L., Wagner J. L., Penick G. D. Orthotopic liver transplantation in canine hemophilia B. Am J Physiol. 1974 Mar;226(3):496–500. doi: 10.1152/ajplegacy.1974.226.3.496. [DOI] [PubMed] [Google Scholar]
  17. Wright T. L., Donegan E., Hsu H. H., Ferrell L., Lake J. R., Kim M., Combs C., Fennessy S., Roberts J. P., Ascher N. L. Recurrent and acquired hepatitis C viral infection in liver transplant recipients. Gastroenterology. 1992 Jul;103(1):317–322. doi: 10.1016/0016-5085(92)91129-r. [DOI] [PubMed] [Google Scholar]

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