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The prevalence of auto antibodies in thymus derived cell bone marrow derived cell and phagocytic immuno deficiency disorders


, : The prevalence of auto antibodies in thymus derived cell bone marrow derived cell and phagocytic immuno deficiency disorders. Clinical Immunology & Immunopathology 14(4): 456-466

Patients with a spectrum of T[thymus-derived]-cell, B[bone marrow-derived]-cell and phagocytic disorders were studied to correlate the specific immunodeficiency associated with autoantibody [auto-Ab] and autoimmune disease. Disorders investigated included X-linked hypogammaglobulinemia, common variable onset hypogammaglobulinemia, selective Ig[immunoglobulin]A deficiency, cellular immunodeficiency with abnormal Ig synthesis, Wiskott-Aldrich syndrome, ataxia telangiectasia, chronic mucocutaneous candidiasis, severe combined immunodeficiency [SCID], chronic granulomatous disease [CGD] and female carriers of CGD. Auto-Ab was detected in all groups except X-linked hypogammaglobulinemia. Clinical autoimmune disease was observed in patients with X-linked hypogammaglobulinemia, common variable onset hypogammaglobulinemia, selective IgA deficiency, Wiskott-Aldrich syndrome, chronic mucocutaneous candidiasis, CGD and female carriers of CGD. No auto-Ab were found in only one immunodeficiency disorder, X-linked hypogammaglobulinemia. Partial B cell dysfunction, T cell dysfunction and phagocytic dysfunction pre-dispose to auto-Ab formation. Clinical autoimmune disorders were found in all types of immunodeficiency except SCID. Patients with partial deficiencies of B and T cell immunity may have regulator cell defects of helper vs. suppressor cell populations which may result in enhanced auto-Ab formation. Of the patients with cellular immunodeficiency and abnormal Ig synthesis who were initially negative for auto-Ab, 2 developed positive auto-Ab after partial reconstruction of T cell function following treatment with bovine thymosin fraction 5 and fetal thymus transplantation. Under certain circumstances, enhanced T cell function may result in auto-Ab formation.

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