{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,13]],"date-time":"2026-07-13T18:41:01Z","timestamp":1783968061554,"version":"3.55.0"},"reference-count":46,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2013,5,15]],"date-time":"2013-05-15T00:00:00Z","timestamp":1368576000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Hepatology Research"],"published-print":{"date-parts":[[2013,9]]},"abstract":"<jats:p><jats:styled-content style=\"fixed-case\">F<\/jats:styled-content>anconi anemia (<jats:styled-content style=\"fixed-case\">FA<\/jats:styled-content>) is an inherited bone marrow failure syndrome due to defective <jats:styled-content style=\"fixed-case\">DNA<\/jats:styled-content> inter\u2010strand cross\u2010link repair. Hematopoietic stem cell transplantation (<jats:styled-content style=\"fixed-case\">HSCT<\/jats:styled-content>) is curative for pancytopenia, but may not prevent the development of non\u2010hematological malignancies. We describe a 26\u2010year\u2010old male patient with <jats:styled-content style=\"fixed-case\">FA<\/jats:styled-content> and Marfan syndrome who in 1994 underwent successful <jats:styled-content style=\"fixed-case\">HSCT<\/jats:styled-content> with bone marrow stem cells from his human leukocyte antigen (<jats:styled-content style=\"fixed-case\">HLA<\/jats:styled-content>)\u2010identical sister. In 2006, three lesions in the liver were detected and resected. The three lesions all showed activation of the \u03b2\u2010catenin pathway and were histologically characterized by a highly differentiated steatotic hepatocellular carcinoma (<jats:styled-content style=\"fixed-case\">HCC<\/jats:styled-content>) with remnants of the underlying adenoma from which it arose, a hepatocellular adenoma with foci of well\u2010differentiated <jats:styled-content style=\"fixed-case\">HCC<\/jats:styled-content>, and a cholestatic adenoma. Risk factors for the emergence of <jats:styled-content style=\"fixed-case\">HCC<\/jats:styled-content> included <jats:styled-content style=\"fixed-case\">FA<\/jats:styled-content> itself, the use of androgens for a period of 3 years preceding <jats:styled-content style=\"fixed-case\">HSCT<\/jats:styled-content> and toxicity of the conditioning regimen. Because of the danger of developing additional <jats:styled-content style=\"fixed-case\">HCC<\/jats:styled-content>, liver transplantation was proposed, taking into consideration that immunosuppression would increase the risk of other malignancies. By using part of the liver of the sister, who already acted as bone marrow donor 13 years earlier, immunosuppression could be avoided. Liver transplantation was performed in 2007 without complication. Five years after liver transplantation the patient is doing well. This case is twofold special being the first case reporting <jats:styled-content style=\"fixed-case\">FA<\/jats:styled-content> co\u2010occurring with <jats:styled-content style=\"fixed-case\">M<\/jats:styled-content>arfan syndrome and being the first reported case of <jats:styled-content style=\"fixed-case\">FA<\/jats:styled-content> treated for <jats:styled-content style=\"fixed-case\">HCC<\/jats:styled-content> by liver transplantation from a <jats:styled-content style=\"fixed-case\">HLA<\/jats:styled-content>\u2010identical sibling donor without the use of immunosuppression.<\/jats:p>","DOI":"10.1111\/hepr.12043","type":"journal-article","created":{"date-parts":[[2013,5,16]],"date-time":"2013-05-16T03:45:25Z","timestamp":1368675925000},"page":"991-998","source":"Crossref","is-referenced-by-count":9,"title":["Living related liver transplantation in an adult patient with hepatocellular adenoma and carcinoma 13 years after bone marrow transplantation for <scp>F<\/scp>anconi anemia: A case report"],"prefix":"10.1111","volume":"43","author":[{"given":"Isabelle","family":"Colle","sequence":"first","affiliation":[{"name":"Department of Hepatology and Gastroenterology Ghent University Hospital  Ghent Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Genevi\u00e8ve","family":"Laureys","sequence":"additional","affiliation":[{"name":"Department of Pediatric Hematology, Oncology and Stem Cell Transplantation Ghent University Hospital  Ghent Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarah","family":"Raevens","sequence":"additional","affiliation":[{"name":"Department of Hepatology and Gastroenterology Ghent University Hospital  Ghent Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Louis","family":"Libbrecht","sequence":"additional","affiliation":[{"name":"Department of Pathology Ghent University Hospital  Ghent Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juliaan G","family":"Leroy","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Ghent University Hospital  Ghent Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Koen","family":"Reyntjens","sequence":"additional","affiliation":[{"name":"Department of Anesthesiology Ghent University Hospital  Ghent Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anja","family":"Geerts","sequence":"additional","affiliation":[{"name":"Department of Hepatology and Gastroenterology Ghent University Hospital  Ghent Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xavier","family":"Rogiers","sequence":"additional","affiliation":[{"name":"Department of General and Hepatobiliary Surgery Liver Transplantation Service Ghent University Hospital  Ghent Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roberto I","family":"Troisi","sequence":"additional","affiliation":[{"name":"Department of General and Hepatobiliary Surgery Liver Transplantation Service Ghent University Hospital  Ghent Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Holger","family":"Hoehn","sequence":"additional","affiliation":[{"name":"Department of Human Genetics University of Wuerzburg  Wuerzburg"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Detlev","family":"Schindler","sequence":"additional","affiliation":[{"name":"Department of Human Genetics University of Wuerzburg  Wuerzburg"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Helmut","family":"Hanenberg","sequence":"additional","affiliation":[{"name":"ENT (HNO) Department University of D\u00fcsseldorf  D\u00fcsseldorf Germany"},{"name":"Department of Pediatrics Wells Center for Pediatric Research Indiana University School of Medicine  Indianapolis Indiana USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vincent","family":"De 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