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25 Cards in this Set
- Front
- Back
What are some of the causes of immunodeficiencies
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Infectious agents, genetic defect, malnutrition, neoplasia, iatrogenic, toxins
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How can immunodeficiency etiologies be classified
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Primary (genetic) and secondary (all the others)
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What are the genetics of primary immunodeficiencies
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Many caused by recessive gene defects, and can be mapped to the X chromosome
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How is B cell function evaluated
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Measuring concentration in blood, Ig levels, and antibody response to pokeweed mitogen
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How is T cell function evaluated
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Concentration in blood, Skin test DTH, Proliferation in response to antigens
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How is phagocyte function evaluated
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Nitro blue tetrazolium uptake, blood concentration
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What are the characteristics of Severe combined immunodeficiency
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B cells and T cells are affected; rare; history of recurrent infections; increased susceptibility to a broad range of pathogens
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Give three examples of Severe combined immunodeficiency (SCID)
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Adenosine Deaminase deficiency, X-linked SCID, Bare lymphocyte syndrome
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Describe adenosine deaminase deficiency
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50% of SCID patients, Autosomal recessive; Toxic dATP accumlates-lymphocytes are especially sensitive
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Describe X-linked SCID
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Recessive mutation in the common gamma chain, on the X chromosome; B+ T-NK-; impaired humoral immunity because of no T cell help
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Describe bare lymphocyte syndrome
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Autosomal recessive; Inability to express MHC class II; reduced numbers of CD4 T cells, so no DTH responses or T-dependent responses
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What are the characteristics of B cell immunodeficiencies
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Specific abnormality of B cell development; susceptible to encapsulated bacteria and enteric infections (lack of IgA)
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What are three examples of B cell immunodeficiencies
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X-linked Agammaglobulinemia; X-linked Hyper-IgM Syndrome; Common variable immunodeficiency
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Describe X-linked Agammaglobulinemia
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Mutation in a tyrosine kinase required for B-cell development in bone marrow; B-T+NK+; no germinal centers or plasma cells; non-random inactivation of X chromosome in B cells of female carriers
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Describe X-linked Hyper-IgM Syndrome
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Defective Ig class switching; Susceptiblility to bacteria and enteric infections; Mutation in CD40L, so defective T Cell help; random pattern of X chromosome inactivation
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Describe Common Variable Immunodeficiency
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Deficit in plasma cell differentiation, Ig deficiency
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What are the characteristics of T cell immunodeficiencies
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Deficits in cell-mediated immunity, and increased susceptibility to viruses and intracellular bacteria
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Describe Congenital Thymic Aplasia (DiGeorge Syndrome or anomaly)
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Hypoplasia or aplasia of the thymus, so T cells are reduced or absent
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Give 4 examples of defects in innate immunity
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Leukocyte adhesion deficiency type 1; Chediak-Higashi syndrome; Chronic granulomatous disease; Complement deficiencies
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Describe Leukocyte adhesion deficiency Type 1 (LAD-1)
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Mutation in LFA-1 and Mac-1, leads to reduced adherence to endothelium, poor chemotaxis, phagocytosis, cytotoxicity, and lymphocyte APC interactions; autosomal recessive
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Describe Chediak-Higashi syndrome
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Phagocyte abnormality-increased fusion of lysosomes resulting in giant cytoplasmic granules
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Describe chronic granulomatous disease
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Phagocyte abnormality affecting components of phagocyte oxidase. Nitroblue tetrazolium dye is used to test phagocyte oxidase activity; Because bacteria and fungi are not controlled, chronic activation of T cells and macrophages results
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Describe complement deficiencies
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C3 deficiency results in frequent and serious pyogenic bacterial infections
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Describe Wiskott-Aldrich Syndrome
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X linked recessive; loss of WASP, resulting in disruption of neutrophil, macrophage, lymphocyte and platelet trafficking; progressive and non-treatable
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Describe Ataxia-telangiectasia
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Autosomal recessive, involved with DNA repair. May affect both T and B cells, Thymus may be hypoplastic
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