Liver transplantation for hereditary hemochromatosis

Abstract

Although hereditary hemochromatosis (HHC) is relatively common, it is an uncommon indication for orthotopic liver transplantation (OLT).O rthotopic liver transplantation (OLT) is the preferred treatment for many patients with complications of end-stage liver disease. The 5-year patient survival rate approaches 70% in many centers. Although it is relatively common in whites, hereditary hemochromatosis (HHC) is an uncommon indication for OLT. In addition, survival of patients who undergo OLT for HHC is markedly reduced compared with most other indications. The recent discovery of the HFE gene has provided a valuable opportunity to refine the diagnosis of HHC in patients with end-stage liver disease and iron overload. OLT also provides a unique opportunity to study the pathogenesis of iron overload. This review summarizes OLT in patients with HHC and iron overload.

Recurrence of non-viral liver disease after orthotopic liver transplantation

“…Liver transplantation is indicated in patients with haemochromatosis with decompensated liver disease and/or hepatocellular carcinoma (123). Survival rates at 1, 3 and 5 years post-transplantation were 72, 62 and 55% respectively (127).…”

“…Hereditary haemochromatosis, an autosomal recessive disease, has a carrier rate of 10% and a prevalence of 0.5% (123). It is characterized by inappropriate absorption and deposition of dietary iron that may result in widespread tissue deposition and cause cirrhosis, hepatocellular carcinoma, cardiomyopathy, DM, and pituitary dysfunction, arthritis, skin pigmentation and cardiac arrhythmias (124).…”

Histopathological Syndromes of Liver Allograft Rejection and Recurrent Disease

“…Familial hypercholesterolemia [233] Hemochromatosis or (inadvertent transplantation of donor with hemochromatosis) (reviewed in [268]) Glycogen storage disease, types I and Ib, III, IV [234,269,270] Hemophilia A and B [233] Niemann-Pick disease [233,234] Hemochromatosis, neonatal (reviewed in [234]) Oxaluria, type I [233,234] Sea-blue histiocyte syndrome [271] Mitochondrial defects, limited to liver (reviewed in [234])…”

Rezidiv der Grunderkrankung nach Lebertransplantation

“…Weiterführende Studien werden klären, ob diese Biomarker zur Prognosestellung für den Langzeitverlauf bei dieser Patientengruppe sinnvoll sind. Eine rezente Arbeit konnte zeigen, dass Patienten mit neuroendokrinen Tumoren dann geeignete Transplantatempfänger sind, wenn sie < 50 Jahre sind, wenn der Primärtumor im Gastrointestinaltrakt gelegen ist, über das portalvenöse System drainiert wird und vollständig entfernt wurde und wenn die Tumorerkrankung für wenigstens 6 Monate vor Transplantation stabil ist [125].…”

“…Ähnliche Resultate liefert die UNOS-Datenbank bei Patienten mit hereditärer Hämochromatose und sekundärer Eisenüberladung im Vergleich zu anderen Erkrankungen (Organ Procurement and Transplantation Network [OPTN], http://www.optn. org/latestData/viewDataReports.asp) [125]. Die Ursachen für die schlechtere Prognose liegen in kardialen Komplikationen, erhöhten postoperativen Infektionsraten, aber auch in einem Rezidiv eines HCC, welches bei bis zu einem Viertel der Patienten mit Hämo-chromatose zum Zeitpunkt der Transplantation nachweisbar ist.…”