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23 Cards in this Set

  • Front
  • Back
Decreased production of B CELLS
=Bruton's Agammaglobulinemia

**X-linked RECESSIVE = occurs in males
=defect in tyrosine kinase gene --> low levels of all classes of immunoglobulins

**Associated w/ recurrent BACTERIAL infections after 6mo of age --> i.e. when levels of maternal IgG antibody decline
Decreased production of T CELLS
=DiGeorge Syndrome (thymic aplasia)

**22q11 DELETION
=thymus and parathyroids fail to form BECAUSE the 3rd and 4th pharyngeal pouch didn't form
**Recurrent viral and fungal infections due to T-cell deficiency

**TETANY = hypocalcemia

**Congenital defects of the heart and great vessels
Decreased production of T AND B CELLS
=Severe Combined Immunodeficiency (SCID)

**Defect in early stem-cell differentiation

=failure to synthesize MHC II antigens
=defective IL-2 receptors
=adenosine deaminase deficiency
How does it present?
**Recurrent viral, bacterial, fungal, and protozoal infections
Decreased ACTIVATION of T Cells
=i.e. IL-12 receptor deficiency

**presents w/ disseminated MYCOBACTERIAL infections
Decreased ACTIVATION of B-Cells

=Hyper-IgM Syndrome
**Defect in CD40 ligand on CD4 T helper cells leads to the INABILITY to class switch

THUS, high levels of IgM, VERY low levels of IgG, IgA, and IgE
**Presents early in life w/ severe PYOGENIC infections
Decreased ACTIVATION of B-Cells

=Wiskott-Aldrich Syndrome
**X-LINKED defect
=can't mount an IgM response to CAPSULAR POLYSACCHARIDES of bacteria

**Associated with:
=elevated IgA levels
=normal IgE levels
=LOW IgM levels
Triad of Symptoms:

1) Recurrent pyogenic INFECTIONS

2) Thrombocytopenic PURPURA

Decreased ACTIVATION of Macrophages
**Job's Syndrome

=failure of gamma-interferon production by helper T-cells
=neutrophils fail to respond to chemotactic stimuli

=reccurent "cold" (noninflammed) staph abscesses


=coarse facies

=retained primary teeth
Phagocytic Cell Deficiency
1) Leukocyte Adhesion Deficiency Syndrome

2) Chediak-Higashi Disease

3) Chronic Granulomatous Disease
Leukocyte Adhesion Deficiency Syndrome
**Defect in LFA-1 adhesion proteins on phagocytes

=presents w/ early severe pyogenic and fungal infections and DELAYED separation of umbilicus

=defect in microtubular function and lysosomal emptying of phagocytic cells
**Recurrent infections by staph and strep

**Partial albinism

**Peripheral neuropathy
Chronic Granulomatous Disease
**Defect in phagocytosis of neutrophils owing to LACK of NADPH oxidase activity
**Marked susceptibility to opportunistic infections with bacteria, esp:

=S. aureus
=E. coli
Diagnosis confirmed by?
**negative nitroblue tetrazolium dye reduction test
Chronic Mucocutaneous Candidiasis
**T-cell dysfunction specifically against Candida albicans

=presents w/ skin and muccous membrane Candida infections
Selective Immunoglobulin Deficiency
**Deficiency in a specific CLASS of immunoglobulins

=possibly due to a defect in CLASS SWITCHING

**Selective IgA deficiency is the MOST common
=sinus and lung infections
=milk allergies and diarrhea common
**Defect in DNA repair enzymes w/ associated IgA deficiency

**Presents w/ CEREBELLAR problems (ataxia) and SPIDER ANGIOMAS (telangiectasia)
Common Variable Immunodeficiency
**Normal numbers of circulating B cells

=decreased plasma cells
=decreased Ig

**Can be acquired in 20s-30s