Long-term outcome of liver transplantation in HCV/HIV coinfected haemophilia patients

G. Goldmann; H. Zeitler; N. Marquardt; S. Horneff; Z. Balta; C. P. Strassburg; J. Oldenburg

doi:10.5482/HAMO-14-07-0027

Hämostaseologie, Inhaltsverzeichnis

Hamostaseologie 2015; 35(02): 175-180
DOI: 10.5482/HAMO-14-07-0027

Original article

Schattauer GmbH

Long-term outcome of liver transplantation in HCV/HIV coinfected haemophilia patients

A single centre study of 10 patientsLangzeit-Ergebnisse nach Lebertransplantation bei HCV/HIV-koinfizierten Hämophilie-PatientenEine Studie mit 10 Patienten

G. Goldmann^*

¹Institute of Experimental Haematology and Transfusion Medicine, University of Bonn

,

H. Zeitler^*

²Centre of Extracorporeal Therapy and Autoimmunity (CETA), Internal Medical Clinic I of the University of Bonn, Germany

,

N. Marquardt

¹Institute of Experimental Haematology and Transfusion Medicine, University of Bonn

,

S. Horneff

¹Institute of Experimental Haematology and Transfusion Medicine, University of Bonn

,

Z. Balta

²Centre of Extracorporeal Therapy and Autoimmunity (CETA), Internal Medical Clinic I of the University of Bonn, Germany

,

C. P. Strassburg

²Centre of Extracorporeal Therapy and Autoimmunity (CETA), Internal Medical Clinic I of the University of Bonn, Germany

,

J. Oldenburg

¹Institute of Experimental Haematology and Transfusion Medicine, University of Bonn

› Institutsangaben

Abstract

Summary

The outcome and clinical features during long term follow-up of 10 haemophilia patients (haemophilia A n = 9, haemophilia B n = 1), who underwent successful orthotopic liver transplantation (OLT) due to hepatitis associated liver disease, are summarised.

Patients

Eight patients were HIV/HCV co-infected. Despite severe postoperative complications, which were not bleeding-associated, all patients survived OLT.

Results

Long-term survival was 70% after in mean 8 years follow- up. Twelve years after OLT one patient developed a cyclosporine-induced nephropathy requiring haemodialysis. HIV-HAART was initiated in all patients after OLT, and allowed a successful HCV treatment in 6 patients. Factor VIII production was sufficient in mean 72 h after OLT and remained stable at subnormal to normal FVIII levels of in median 30% (range 14–96%) also during long-term follow-up. Post-OLT spontaneous bleeding events were rare compared to pre-OLT, therefore, the performance status improved in all patients.

Discussion

OLT substitutes the hepatic FVIII but has no effect on the extrahepatic endothelial FVIII production, suggesting that in case of severe tissue injury enhanced bleeding might occur. Additionally, after OLT there is no acute phase reaction of the FVIII protein. Therefore, our OLT patients received in case of a reduced FVIII activity a peri-interventional prophylactic short-term FVIII substitution in surgical and diagnostic interventions with high bleeding risk.

Conclusion

Bleeding and wound healing disturbances were not seen.

Zusammenfassung

Wir berichten über 10 Hämophilie-Patienten (Hämophilie A n = 9, Hämophilie B n = 1), die sich aufgrund einer viral bedingten schwersten Lebererkrankung erfolgreich einer Lebertransplantation (OLT) unterzogen hatten.

Patienten

Bei 8 Patienten bestand eine HCV/ HIV-Koinfektion.

Ergebnisse

Trotz z. T. schwer wiegender postoperativer Komplikationen, die nicht blutungsassoziiert waren, überlebten alle Patienten den primären Eingriff der Transplantation. Im Langzeit-Follow-up über im Median 8 Jahre betrug das Überleben in unserem Kollektiv 70%. Ein Patient entwickelte ein Cyclosporin-induziertes Nierenversagen und wurde 12 Jahre nach OLT dialysepflichtig. Die HCV-Therapie war nach Initiierung der HIV- HAART post-OLT in 6 Fällen erfolgreich. In allen Fällen erreichte die Transplantation postoperativ nach im Median 72 h einen subnormalen bis normalen FVIII-Wert von im Median 30% (Spannbreite: 14–96%), der im weiteren Verlauf stabil blieb. Die Anzahl spontaner Blutungsereignisse ging deutlich zurück, entsprechend verbesserte sich der Mobilitätsstatus aller Patienten.

Diskussion

Die OLT ersetzt lediglich den hepatisch produzierten FVIII, sodass Blutungskomplikationen auch nach erfolgreicher OLT bei größeren Gefäß- und Gewebetraumata möglich sind. Insbesondere fehlt nach OLT die Akutphase-Eigenschaft des FVIII. In unserem Kollektiv wurde, sofern der FVIII-Wert unterhalb des Referenzbereichs lag, bei allen blutungsgefährdeten Eingriffen prophylaktisch perinterventionell eine kurzfristige FVIII-Substitutionstherapie durchgeführt.

Schlussfolgerung

Blutungskomplikationen oder Wundheilungsstörungen traten nicht auf.

Keywords

Haemophilia - liver transplantation - hepatitis - factor VIII - inhibitor

Schlüsselwörter

Hämophilie - Lebertransplantation - Hepatitis - Faktor VIII - Hemmkörper

Volltext

Referenzen

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