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19 Cards in this Set

  • Front
  • Back
Describe Bruton's agammaglobulinemia
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
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)
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
T cell dysfunction specifically against Candida albicans

presents with skin and mucou membrane Candida infections
Decribe Severe combined immunodeficiency (SCID)
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
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
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
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
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
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
Phagocytic deficiency

Defect in LFA-1 adhesion protiens on phagocytes

presents early in life with many pyogenic infections
Describe Hyper-IgM syndrome
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
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. Anticentromere
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
antibody mediated

due to the presence of preformed anti-donor antibodies in the transplant recipient

occurs within minutes after the transplantation
Describe Acute Rejection
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
Antibody mediated vascular damage (fibrinoid necrosis)

occurs months to years after transplantation

Describe Graft vs Host Disease
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