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23 Cards in this Set

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