Pathogenesis of Immunodeficiency Disorders and Gastrointestinal Lesions Ichiro Hirata 1 1Department of Internal Medicine II, Osaka Medical College Keyword: 原発性免疫不全症 , 続発性免疫不全症 , 消化管病変 , 自己免疫疾患 , 悪性腫瘍 pp.1094-1104
Published Date 2005/7/25
DOI https://doi.org/10.11477/mf.1403100134
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 Primary immunodeficiency disorders (PID) which demonstrate organic lesions in the digestive tracts are CVID, XLA, IgA deficiency, SCID, Wiscott-Aldrich syndrome, Ataxia-Telangiectasia, Chronic granulomatous disease, Chediak-Higashi syndrome,Anhidrotic Ectodermal Dysplasia and Hyper IgE syndrome. Various gastrointestinal (GI) lesions in PID which have been reported are GI infections, sprue-like disorder, various inflammatory lesions including ulcers, autoimmune disorders (IBD, PA) and GI tumors (cancer, lymphoma). The morphology of most these lesions might be mainly influenced by repeated and prolonged severe GI infection. Causal diseases of secondary immunodeficiency disorders are AIDS, ATL/L, GvHD, collagen disease, α-chain disease and iatrogenic factors (immunosuppressive agents, radiation, anticancer drug etc.) The GI lesions of secondary immunodeficiency disorders are due to opportunitic infection of parasites and virus, apoptosis of tissues, Kaposi sarcoma and infiltration of the GI mucosa by primary tumors.

 It is necessary to examine a patient carefully and undertake an immunological evaluation of the patient when lesions with unusual morphology have seen.

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