The immunodeficiency and the self-immunity are two poles the immune response that may have interrelation each other with some frequency. In present paper are showed the autoimmune syndromes caused by primary immunodeficiencies. Also, are described other primary immunodeficiencies including common variable immunodeficiency, the hyper Ig M syndrome, the selective of Ig A deficiency, the hypogammaglobulinemia linked to X chromosome, the Digeorge's syndrome, the chronic granulomatous diseases, the Wiskott Aldrich's syndrome, and the complement system defects, those that with a variable frequency may be associated with self-immune manifestations, mainly the hematologic ones. The immuno-pathogen leading mechanism is the persistent antigenic stimulation due to a immune system defect to remove pathogens; also, are included the regulatory T cells defects, deregulation in homeostatic proliferation secondary to linphopenia and genetic factors.
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