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18 Cards in this Set
- Front
- Back
- 3rd side (hint)
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Immunodeficiency Disease List (13)
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Leukocyte Adhesion Deficiency (CD18)
Chronic Granulomatous Disease Myeloma Lymphoma SCID (autosomal x linked) Agammaglobulinemias (x linked, common variable Selective IgA deficiency x linked hyper IgM activation induced cytidine daminase deficiency Digeorge's syndrome mucocutaneous candidiasis AIDS |
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Mucocutaneous candidiasis
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Dysfunction of cell mediated immunity
associated with yeast (candida) infections. |
other T cell functions appear normal
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Digeorge's Syndrome
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congenital thymic aplasia due to abnormal development at 12th week.
face appears odd Pre T cells present B cells present but cannot be regulated due to lack of T cells |
striking facial features
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Selective IgA deficiency
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Most common immunodeficiency (1 in 400) most asymptomatic
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X linked Hyper IgM syndrome
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Defective CD40 ligand causes accumulation of B cells unable to get to IgG or other isotypes.
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Lymph nodes may lack germinal centers
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SCID Autosomal
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50%- ADA Deficient or Purine Phosphorylase PNP Deficient
Bare-Leukocyte Syndrome? TAP genes? RAG deficiencies? |
ADA PNP- toxic buildup
Bare Leukocyte- Class I II HLA molecules T cell subsets TAP- CD* Antigen Presentation RAG- gene rearrangement |
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SCID x linked
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Deficient cytokines (IL 2, 4,7,9,15) IL-7 key
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Complement abnormalities
Phagocytic dysfunction Lymphocyte deficiencies individual what are the percents? |
Complement 2-4
Phagocytic -15 B cell- 50 T cell 30 |
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leukocyte adhesion deficiency
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defective integrin CD18
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can't move from blood to infection
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Chronic Granulomatous Disease
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Defective superoxide production
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can't destroy bacteria
fails to reduce nitroblue tetrazolium dye |
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Agammaglobulinemias
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B cells are missing or dysfunctional
may be because of T helper faulty regulation |
Xlinked congenital
Common variable immunodefcieiency Selective IgA X linked hyper IgM activation induced cytidine deaminase AID- can't class switch |
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X linked Congenital Agammaglobulinemia
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(Bruton's) defective tyrosine kinase (btk) necessary for joining of V to DJ. Pre B cells present but others not
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common in young boys
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Common Variable immunodeficiency
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CVI resembles X linked
older patients B cells present, autosomal poor regulatory signaling defects |
older populations
even between men and women |
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AIDS
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from HIV
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Impairment of CD4+ inducer T cells
Dendritic cells Macrophages (CD4 entry) Gp120 binds to CD4 CCR5 T cell macrophage can loose humoral and cellular immunity Kaposi's Sarcoma CXCR4 T cell only |
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Monitoring HIV
false positives? false negatives? |
false positive- western blot
neg- kinetics and time of HIV infection Test for genome with PCR Test for circulating CD4 cells( immunofluorescence with monoclonal antibody landmarks?? |
CD4 averages
-1200/uL normal -800 HIV asymptomatic -500 first oppurtunistic infection less than 200 AIDS |
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Functions of CD4 Cells (6)
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CTL activation
NK amplify B cell activation Antibody Class Switching Direct Effector Macrophage Activation |
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Cytokines for Th1 subset
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Inflammatory
IL-2 IFN gamma TNF alpha |
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Cytokines for Th2
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IL-4
IL-5 IL-10 |
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