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IMMUNODEFICIENCY STATE (IMMUNOSUPPRESSION) |
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immunoglobulin deficiency (defects in humoral immunity) |
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1� immunoglobulin deficiency: Bruton's agammaglobulinaemia (X-linked recessive) transient hypogammaglobulinaemia in childhood late-onset 1� hypogammaglobulinaemia functional antibody deficiency (normal numbers of T and B lymphocytes) thymoma + hypogammaglobulinaemia isolated IgA deficiency isolated IgG subclass deficiencies isolated IgM deficiency IgG and IgA deficiency with raised IgM |
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2� immunoglobulin deficiency: premature babies uraemia and other sever metabolic disturbances (abnormal protein synthesis) drugs, eg. phenytoin, penicillamine, diazoxide severe nephrotic syndrome (loss in urine) thoracic duct fistula paroxysmal nocturnal haemoglobinuria dystrophia myotonica |
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deficient cellular immunity |
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defective T lymphocyte function: thymic aplasia (normal parathyroids in Nezelof syndrome; with hypoparathyroidism in DiGeorge's syndrome) severe combined immunodeficiency ('SCID') [Swiss type immunodeficiency; decreased T and B lymphocytes; no specific Ig production] purine nucleoside phosphorylase deficiency (� abnormal red cells) ataxia-telangiectasia [autosomal recessive; IgA deficiency + cerebellar ataxia + oculo-cutaneous telangiectasia] Wiscott-Aldrich syndrome [X-linked recessive; ?faulty antigen presentation by macrophages; thrombocytopaenia + eczema; absent antibody production against polysaccharide antigens] Bloom's syndrome [low Ig levels; lymphomas occur] |
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defective phagocyte function: Job's syndrome [defective neutrophil chemotaxis; raised IgE] chronic granulomatous disease [X-linked; impaired intracellular killing of catalase positive organisms by PMN's] Chédiak-Higashi syndrome [associated with albinism; impaired killing due to defective neutrophil lysosomal formation] myeloperoxidase deficiency [autosomal recessive; neutrophil and monocyte myeloperoxidase low or absent] |
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complement deficiencies |
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C1 esterase inhibitor deficiency [hereditary angio-oedema; autosomal dominant] C1q deficiency [discoid lupus] C1q, C1r, C4 or C2 deficiency [increased risk of immune-complex vasculitis] C3 or C3b inhibitor deficiency [associated with Klinefelter's syndrome; causes exhaustion of alternative pathway] C5, C6, C7 or C8 deficiency [recurrent Neisseria infections] |
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causes of 2� immunodeficiency (various mechanisms involved) |
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diabetes mellitus HIV infection (CD4 cells affected) sarcoidosis lymphomas hyposplenism / splenectomy following leprosy, tuberculosis or measles severe malnutrition extremes of age any severe intercurrent illness drugs, especially steroids and cytotoxics radiation |
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