Cholangiopathy and tumors of the pancreas, liver, and biliary tree in boys with X-linked immunodeficiency with hyper-IgM.

@article{Hayward1997CholangiopathyAT,
  title={Cholangiopathy and tumors of the pancreas, liver, and biliary tree in boys with X-linked immunodeficiency with hyper-IgM.},
  author={Anthony R. Hayward and Jack Levy and Fabio F. Facchetti and Luigi D Notarangelo and Hans D. Ochs and Amos Etzioni and Jean Yves Bonnefoy and Mary Cosyns and Aaron B Weinberg},
  journal={Journal of immunology},
  year={1997},
  volume={158 2},
  pages={977-83}
}
We report an association between X-linked immunodeficiency with hyper-IgM (XHIM) and carcinomas affecting the liver, pancreas, biliary tree, and associated neuroectodermal endocrine cells. The tumors were fatal in eight of nine cases and in most instances were preceded by chronic cholangiopathy and/or cirrhosis. An additional group of subjects with XHIM had chronic inflammation of the liver or bile ducts but no malignancy. Many patients with XHIM were infected with cryptosporidia. CD40 is… CONTINUE READING
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