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8 Cards in this Set
- Front
- Back
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Burton's agammaglobulinemia
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X-linked recessive
defect in BTK --> blocks B cell differentiation/maturation Present: recurrent bactrial infections after 6 mo due to opsonization defect Labs: nl pro-B, dec maturation, # of B cells, all immunoglobulins |
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Hyper IgM
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-Defective CD40L on helper T cell- inability to class switch
-severe pyogenic infections early in life labs: inc IgM, dec G, A, E |
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Selective Ig Deficiency
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-defect in isotype switching
-sinus and lung infections, milk allergie, diahrrea, anaphylaxis on exposure to blood products w/ IgA labs: IgA deficiency more common, failure to mature into plasma, dec secretory IgA |
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Common Variable Immunodeficiency
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-defect in b cell maturation
-aquired in 20-30s, inc risk of autoimmune dz, lymphoma, sinopulm. infections -labs: nl # of B cells, dec Plasma cells and Ig |
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Thypic Aplasia (Digeorge Syndrome)
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-22q11 del, failure to develop 3rd and 4th pharyngeal pouches (missing thymus, inf parathyroid)
-tetany (hypocalcemia), recurrent viral/fungal infections (T cell def), congenital heart and great vessel defects lab: thymus and parathyroid fail to develop --> dec T cells, dec PTH, dec Ca2+. Absent thymic shadow on CXR |
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IL-2 R Deficiency
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- dec Th1 response
- disseminated mycobacterial infections labs: dec IFN-gamma |
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Hyper IgE syndrome (Job's)
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-th cells fail to produce IFN-gamma --> inability of neutrophils to respond to chemotactic stimuli
-FATED: coarse face, cold staph abscesses, retained primary teetch, inc IgE, Eczema lab: inc IgE |
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Chronic Mucocutaneous Candidiasis
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-T cell dysfunction
-candida albicans, infections of skin and mucuos membranes |
