FA Immuno: Immunopathology
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- Describe Bruton's agammaglobulinemia
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B-cell deficiency; X linked recessive defect in a tyrosine kinase gene asscoaited with low levels of all classes of immunoglobins
Associated with recurrent Bacterial infections after 6 months of age, when levels of maternal IgG antibody decline
"Bacterial infxns in Boys" - Describe Selective imunoglobulin deficiency
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deficiency in a specific class of immunoglobulins - possibly due to a defect in isotype switching
Slective IgA deficiency is the most common selective immunoglobulin deficiency
presents with sinus and lung infections - Describe DiGeorge Syndrome (Thymic aplasia)
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thymus and parathyroids fail to develop owing to failure of development if the 3rd and 4th pharyngeal pouches
presents with Tetany owing to hypocalcemia
Recurrant viral anf fungal infections due to T-cell deficiency
congenital defects of the Heart and great vessels - Describe Chronic mucocutaneous candidiasis
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T cell dysfunction specifically against Candida albicans
presents with skin and mucou membrane Candida infections - Decribe Severe combined immunodeficiency (SCID)
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defect in early stem cell differentiation
preent with recurrant viral, bacterial, fungal, and protazoal infections
may have multiple causes (eg, failure to synthesize MHC II antigens, defective IL-2 receptors, or adenosine deaminase deficiency) - Describe Wiskott- Aldrich syndrome
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Deficiency of B and T cells
X-linked defect int he ability to mount an IgM response to capsular polysacchrides of bacteria
associated with elevated IgA levels, normal IgE levels and low IgM levels
Triad of symptoms includes recurrant pyogenic Infections, thrombocytopenic Pupura, Eczema (WIPE) - Describe Ataxia telangiectasia
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Deficiency of both B and T cells
Defect in the repair nzms with associated IgA deficiency
presentc with cerebellar problems (ataxia) and spider angiomas (telangiectasia) - Describe Chronic Granulomatous disease
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phagocytic deficiency
defect in the phagocytosis of neutrophils owing to the lack of NADPH oxidas activity or similar enzymes
presents with marked susceptibility to oppurtunistic infections with bacteria, especially S. aureus and Aspergillus
diagnoses confirmed with negative nitroblue tetrazolium dye reduction test - Describe Chediak-Higashi disease
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autosomal recessive disease, phagocytic deficiency
defect in microtubular function and lysosomal emptying of phagocytic cells
presents with recurring pyogenic infections by staphylococci and streptococci - Describe Job's Syndrome
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phagocytic deficiency
failure of y-interferon production of helper T cells
Neutrophils fail to respond to chemotactic stimuli
presents with recurrent "cold" (non-inflamed) staphyloccocal abscesses, eczema, and high levels of IgE - Describe Leukocyte Adhesion deficiency Syndrome
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Phagocytic deficiency
Defect in LFA-1 adhesion protiens on phagocytes
presents early in life with many pyogenic infections - Describe Hyper-IgM syndrome
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defect in CD40 ligand on CD4 T helper cells lead to instability to calss switch
presents early in life with sever pyogenic infections
high levels IgM, but very low levels of IgG, IgA and IgE - Describe IL-12 receptor deficiency
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phagocytic deficiency
present with disseminated mycobacterial infections -
What disorder is associated with each of the following types of Autoantibodies...?
1. Antinuclear antibodies (ANA)
2. Anti-dsDNA, anti-Smith
3. Antihistone
4. Anti-IgG
5. Antineutrophil cytoplasmic antibodies (ANCA)
6 -
1. SLE
2. speceific for SLE
3. drug induce lupus
4. (rheumatoid factor)Rheumatoid Arthritis
5. vasculitis
6. scleroderma (CREST) -
What disorder is associated with each of the following types of Autoantibodies...?
1. Anti-Scl-70
2. Antimitochondrial
3. Antigliadin
4. Anti-basement membrane
5. Anti-epithelial cell
6. Antimicrosomal -
1. Scleroderma (diffuse)
2. primary biliary cirrhosis
3. celiac disease
4. Goodpasture's syndrome
5. Pemphigus vulgaris
6. Hashimotos Thyroiditis - Describe Hyperacute transplant rejection
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antibody mediated
due to the presence of preformed anti-donor antibodies in the transplant recipient
occurs within minutes after the transplantation - Describe Acute Rejection
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cell mediated due to cytotoxic T lymphocytes reacting against foreign MHCs
occurs within weeks after the transplant
reversible with immunosuppressants such as cyclosporin and OKT3 - Describe Chronic Rejection
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Antibody mediated vascular damage (fibrinoid necrosis)
occurs months to years after transplantation
Irreversible - Describe Graft vs Host Disease
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Grafted immunocompetent T cells proliferate in the irradiated immunocompromised host and reject cells with foreign proteins, resulting in severe organ dysfunction
major symptoms include a maculopapular rash, jaundice, hepatosplenomegaly, and diarrhea