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5 Cards in this Set
- Front
- Back
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Bare lymphocyte syndrome
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SCID
HLA's not expressed Sgs not presented to TH4 T cells, so B cells not activated and cytotoxic cess not generated |
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Wiskott-Aldrich Syndrome
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SCID
X linked disease - B and T cells are functionally abnormal Thrombocytopenia, recurrent bact infections, allergies |
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Ataxia Telangiectasia
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SCID
DNA damage from radiation or oxidative damage Neurological and vessel dilation, lymphopenia, thymic hypoplasia, depressed Ig, possible lymphoid neoplasia |
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Omenn Syndrome
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T+/B- SCID
Reduced or partial RAG activity (Ig rearrangement) skin and GI are infilitrated by eosinophils and activated T cells producitn Th2 cytokines Results in hyper IgE syndrome |
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Congenital Thymic Aplasia / DiGeorge Syndrome
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spontaneous gene mutation caused thymus to develp abnormally
Thymic aplasia, hypoparathyroidism, no T cells |
