title: Liver transplantation for severe hepatitis in patients with common variable immunodeficiency.
abstract: CVID is a heterogeneous group of primary immunodeficiency diseases characterized by hypogammaglobulinemia, recurrent bacterial infections, and frequent autoimmune manifestations. The post-transplant course of liver transplant recipients with CVID is rarely described. We report two patients with CVID complicated by severe enteropathy who underwent living donor liver transplantation for liver failure because of severe hepatitis. The post-transplant course was complicated by recurrent acute rejection, leading to ductopenic rejection in one and recurrent hepatitis in the other. We reviewed the tissue samples histologically and immunohistochemically. Native livers showed submassive hepatocyte necrosis in one and cirrhotic liver with active hepatitis in the other, both with infiltration of CD8+ T cells accompanied by endothelialitis and bile duct damage; the intestine contained increased numbers of intraepithelial CD8+ T cells with apoptosis of epithelial cells. The liver allograft exhibited acute rejection, with prominent CD8+ T cells infiltrating the bile duct or endothelium. In the allograft following the diagnosis of post-transplant recurrent hepatitis, CD8+ T cells comprised the majority of infiltrating cells in portal areas spilling over into hepatic parenchyma. Our cases suggest that T cells contribute to the pathogenesis of CVID in native organs as well as allografts and may constitute evidence of T-cell deregulation in the pathogenesis of CVID.
authors.affiliation: Department of Diagnostic Pathology, Kyoto University Hospital, Kyoto, Japan.
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Murakawa
authors.forename: Aya
authors.initials: A
authors.lastname: Miyagawa-Hayashino
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Ogura
authors.forename: Hiroto
authors.initials: H
authors.lastname: Egawa
authors.forename: Shinya
authors.initials: S
authors.lastname: Okamoto
authors.forename: Yuji
authors.initials: Y
authors.lastname: Soejima
authors.forename: Manabu
authors.initials: M
authors.lastname: Kurosawa
authors.forename: Shinji
authors.initials: S
authors.lastname: Sumiyoshi
authors.forename: Shinji
authors.initials: S
authors.lastname: Uemoto
authors.forename: Hironori
authors.initials: H
authors.lastname: Haga
mesh_terms: D000328:Adult; D001652:Bile Ducts / Q000473:pathology; D018414:CD8-Positive T-Lymphocytes / Q000276:immunology; D002648:Child; D017074:Common Variable Immunodeficiency / Q000150:complications / Q000628:therapy*; D004847:Epithelial Cells / Q000166:cytology; D005220:Fathers; D005260:Female; D005434:Flow Cytometry / Q000379:methods; D006505:Hepatitis / Q000150:complications / Q000601:surgery / Q000628:therapy*; D006801:Humans; D007150:Immunohistochemistry / Q000379:methods; D007410:Intestinal Diseases / Q000150:complications / Q000628:therapy*; D017093:Liver Failure / Q000150:complications / Q000628:therapy*; D016031:Liver Transplantation / Q000379:methods*; D019520:Living Donors; D009035:Mothers; D014184:Transplantation, Homologous; D016896:Treatment Outcome