Liver transplantation for severe hepatitis in patients with common variable immunodeficiency

Yasuhiro Murakawa, Aya Miyagawa-Hayashino, Yasuhiro Ogura, Hiroto Egawa, Shinya Okamoto, Yuji Soejima, Manabu Kurosawa, Shinji Sumiyoshi, Shinji Uemoto, Hironori Haga

研究成果: ジャーナルへの寄稿学術誌査読

17 被引用数 (Scopus)

抄録

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.

本文言語英語
ページ(範囲)E210-E216
ジャーナルPediatric Transplantation
16
6
DOI
出版ステータス出版済み - 9月 2012

!!!All Science Journal Classification (ASJC) codes

  • 小児科学、周産期医学および子どもの健康
  • 移植

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