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23 Cards in this Set
- Front
- Back
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Deficient tyrosine kinase blocks B cell maturation
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X-linked Bruton's hypogammaglobulinemia
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Recurrent pyogenic infections after 6 months of age (respiratory strep pneumo and H flu) and low immunoglobulins of all classes, no circulating B cells
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X-linked Bruton's hypogammaglobulinemia - note it is the most common
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B-cell maturation defect, hypogammaglobulinemia, affects both sexes, recurrent bacterial infections, increased susceptibility to Giardia. Increased risk later in life to autoimmune disease, lymphoma, or gastric cancer.
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Common variable immunodeficiency
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Repeated sinopulmonary and gastrointestinal infections. Most common
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IgA deficiency
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Recurrent infections by viral and fungal organisms, hypoparathyroidism
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Di George (failure of 3rd and 4th POUCHES to form)
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Which paramyxovirus results in a T-cell deficiency?
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Measles
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Where is the mutation in Hyper IgM Syndrome?
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CD40L on CD4+ cells
(prevents B cells from class switching) |
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Failure of TAP 1 molecules to transport peptides to the ER?
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MHC class I deficiency. CD8+ cells deficient, but CD4+ normal.
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Possible causes include a defect in tyrosine kinase ZAP-70, adenosine daminase deficiency, defective IL-2 receptors, or failure to synthesize MHC II antigens?
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SCID
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In the X-linked form a SCID there is a defiencey in the receptor for which IL?
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IL-2 (gamma chain of IL-2 receptor)
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Eczema, thrombocytopenia, recurrent infection
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Wiskott-Aldrich
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X-linked, defect in cytoskeletal glycoprotein, depressed IgM, defective response to bacterial polysaccharides, gradual loss of humomral and cellular response.
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Wiskott-Aldrich
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Defect in DNA repair enzymes with associated IgA deficiency?
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Ataxia telangiectasia
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How can you tell the difference between X linked Bruton's agammaglobulinemia and CVID?
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In CVID the number of B cells will be normal but they will be reduced or absent in Bruton's.
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In Wiscott-Aldrech syndrome, what are the immunoglobulin findings?
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Low levels of IgM, elevated IgA and IgG, normal IgE,
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In DiGeorge's syndrome, which is the only type of T cell that may still exist?
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delta-gamma T cells - function unknown. They are the only type that doesn't require education and selection in the thymus.
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Atopic allergy requires (1-4)
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TH2 helper cells (induce isotype switching to IgE)
mast cells, B lymphs, and plasma cells so folks with DiGeorge do not have atopic allergy |
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Diagnosis is made by a negative NBT dye reduction test.
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chronic granulomatous disease
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Defect in phagocytosis of PMNS because they lack NADPH oxidase. Marked susceptibility to opportunistic pathogens, esp. S. aureus, E. coli, and Aspergillus.
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Chronic granulomatous disease
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Defect in microtubular function and lysosomal emptying of phagocytic cells. Recurrent pyogenic staph and strep infections.
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Chediak-Higashi Disease
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TH cells cannot produce INF-gamma --> cold staph abscesses, eczema, high IgE
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Jobs syndrome
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Leukocyte adhesion deficiency syndrome - defect is in:
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LFA-1 adhesion protein on phagocytes.
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Presents with disseminated mycobacterial infections (because they cannot stimulate T cells)
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IL-12 deficiency
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