Abstract

Backgound: Graft versus host disease (GVHD), a common complication of
hematopoietic stem cell transplantation (HSCT), frequently causes liver injury and in some cases leads to an end stage liver disease. Some authors  have suggested that extra hepatic GVHD (skin and intestinal) should be a contraindication to liver transplantation (OLTx) because of a poor survival of these patients. The aim of the study is to present a successful OLTx in two  patients with severe chronic GVHD after HSCT.
Material/Method: First case: 8- years-old boy received MUD HSCT due to CD40
ligand deficiency complicated by chronic Cryptosporidium infection with cholangitis sclerosans. GVHD III/IV degree occured 8 days after HSCT. Vanishing bile duct syndrome caused liver failure. Successful OLTx was performed 11 months later after resolution of skin and intestinal symptoms of GVHD. Second case: 6 month old boy with X-linked severe combined immunodeficiency received MUT HSCT. GVHD III degree occured 26 days after HSCT. Six months later,  when skin and intestinal symptoms had been resolved, living related OLTx was successfully performed due to an end stage liver involvement.
Results: Both patients have good liver function, full immunological reconstitution and haven't GVHD symptoms respectively six months and four years after OLTx. Conclusions: OLTx appears to be an effective treatment for post HSCT GVHD even when both hepatic and extra hepatic symptoms are present.

Keywords: Liver Transplantation, Stem Cell Transplantation, case report