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Immunodeficiencies, Adenosine Deaminase Def. -in 50% of autosomal SCID Purine Nucleotide Phosphorylase Def ->both for purine degradation def->nucleotide metabolites accum. ->toxic to T, B cells Thymus doesn't develop SCID thymus fails to diff'ate, Immunodeficiencies Phagocytic Cell Defects Defect in Extravasation steps: 1.Rolling-lack of sialyl-Lewis-X ->can't bind E/P-selectins 2.Adhesion-integrin defects eg LFA-1, Mac-1, b2 integrins ->LAD syndrome 3.Migration-def. chemotactic gradient -->high WBC count b/c WBCs stay in circ., Immunodeficiencies Phagocytic Cell Defects Defect in Phagocyte fcn Chronic Granulomatous Dz-def. NADPH oxidase ->defect in superoxide production->ox. burst Chediak-Higashi Syn-def. gene in vesicle formation ->def. killing due to def. lysosome/phago fusion, SCID -onset by 6 mo -recurrent infections of all types -X-linked and autosomal forms -absent T and B cell immunity Tx-bone marrow x-plant Thymus Development SCID thymus fails to diff'ate, Immunodeficiencies Mycobacteria TLR-2 on macs ->macs produce IL-12 ->IL-12R on T cells ->T cells release IFN-g ->recruit/activate macs ->form granuloma Defects in any cytokine ->fewer granuloma, less killing, Immunodeficiencies Secondary Immunodefs Arise from other Dzs, iatrogenic, burns, etc Extremely common -from malnutrition->weak immune system Recurrent Infections-Major problem -latent viruses wait for immunosuppression, Immunodeficiencies T cell Defects Chronic Mucocutaneous Candidiasis -male/female, mostly children -skin/mucus memb infections w/ C. albicans -selective T cell defect for Candida -NL T cells for other Ags -NL B cell, Ig levels-AB response NL Tx-anti-fungal agents-poor prognosis, -No circulating ABs or mature B cells in serum -In males b/c X-linked (XLA) -btk (Bruton's Tyr Kinase) gene defective -B cell maturation halted at pre-B cell stage ->btk role to push cell past pre-B stage -Tx-periodic Ig injections -btk not necessary for other cell development Newborn Ig Levels At birth, ~ same levels of IgG as mom -drops after birth->very low 3mo-1yo -baby starts making IgG at 6 mo -IgM production almost immed at birth, Immunodeficiencies ABNL production of Immune components Plasma cell neoplasia -ABNL Ig production ->suscep to infection eg-multiple myeloma -Waldenstrom's macroglobulinemia B, T cell neoplasia -lymphoma, leukemia ->suppress NL fcn ->suscep to infection, SCID -onset by 6 mo -recurrent infections of all types -X-linked and autosomal forms -absent T and B cell immunity Tx-bone marrow x-plant Bone Marrow X-plant Recipient, Donor must share some MHCs Otherwise-> -Host vs. Graft Dz-Mature host T cells kill graft -Graft vs. Host dz->mature graft T cells kill host -deplete donor of mature T cells->success