PMID- 23675868
OWN - NLM
STAT- PubMed-not-MEDLINE
DCOM- 20130916
LR  - 20211021
IS  - 1386-6346 (Print)
IS  - 1386-6346 (Linking)
VI  - 43
IP  - 9
DP  - 2013 Sep
TI  - Living related liver transplantation in an adult patient with hepatocellular 
      adenoma and carcinoma 13 years after bone marrow transplantation for Fanconi 
      anemia: A case report.
PG  - 991-8
LID - 10.1111/hepr.12043 [doi]
AB  - Fanconi anemia (FA) is an inherited bone marrow failure syndrome due to defective 
      DNA inter-strand cross-link repair. Hematopoietic stem cell transplantation 
      (HSCT) is curative for pancytopenia, but may not prevent the development of 
      non-hematological malignancies. We describe a 26-year-old male patient with FA 
      and Marfan syndrome who in 1994 underwent successful HSCT with bone marrow stem 
      cells from his human leukocyte antigen (HLA)-identical sister. In 2006, three 
      lesions in the liver were detected and resected. The three lesions all showed 
      activation of the beta-catenin pathway and were histologically characterized by a 
      highly differentiated steatotic hepatocellular carcinoma (HCC) with remnants of 
      the underlying adenoma from which it arose, a hepatocellular adenoma with foci of 
      well-differentiated HCC, and a cholestatic adenoma. Risk factors for the 
      emergence of HCC included FA itself, the use of androgens for a period of 3 years 
      preceding HSCT and toxicity of the conditioning regimen. Because of the danger of 
      developing additional HCC, 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 FA co-occurring with Marfan syndrome and being the 
      first reported case of FA treated for HCC by liver transplantation from a 
      HLA-identical sibling donor without the use of immunosuppression.
CI  - (c) 2013 The Japan Society of Hepatology.
FAU - Colle, Isabelle
AU  - Colle I
AD  - Department of Hepatology and Gastroenterology, Ghent University Hospital, Ghent, 
      Belgium.
FAU - Laureys, Genevieve
AU  - Laureys G
FAU - Raevens, Sarah
AU  - Raevens S
FAU - Libbrecht, Louis
AU  - Libbrecht L
FAU - Leroy, Juliaan G
AU  - Leroy JG
FAU - Reyntjens, Koen
AU  - Reyntjens K
FAU - Geerts, Anja
AU  - Geerts A
FAU - Rogiers, Xavier
AU  - Rogiers X
FAU - Troisi, Roberto I
AU  - Troisi RI
FAU - Hoehn, Holger
AU  - Hoehn H
FAU - Schindler, Detlev
AU  - Schindler D
FAU - Hanenberg, Helmut
AU  - Hanenberg H
FAU - De Wilde, Vincent
AU  - De Wilde V
FAU - Van Vlierberghe, Hans
AU  - Van Vlierberghe H
LA  - eng
GR  - R01 CA138237/CA/NCI NIH HHS/United States
GR  - R01 CA155294/CA/NCI NIH HHS/United States
PT  - Journal Article
DEP - 20130515
PL  - Netherlands
TA  - Hepatol Res
JT  - Hepatology research : the official journal of the Japan Society of Hepatology
JID - 9711801
PMC - PMC3888060
MID - NIHMS540601
OTO - NOTNLM
OT  - Fanconi anemia
OT  - bone marrow transplantation
OT  - hepatocellular carcinoma
OT  - liver transplantation
COIS- Competing interests The authors declare that they have no competing interests.
EDAT- 2013/05/17 06:00
MHDA- 2013/05/17 06:01
PMCR- 2014/01/10
CRDT- 2013/05/17 06:00
PHST- 2012/09/15 00:00 [received]
PHST- 2012/12/04 00:00 [revised]
PHST- 2012/12/07 00:00 [accepted]
PHST- 2013/05/17 06:00 [entrez]
PHST- 2013/05/17 06:00 [pubmed]
PHST- 2013/05/17 06:01 [medline]
PHST- 2014/01/10 00:00 [pmc-release]
AID - 10.1111/hepr.12043 [doi]
PST - ppublish
SO  - Hepatol Res. 2013 Sep;43(9):991-8. doi: 10.1111/hepr.12043. Epub 2013 May 15.