What is the incidence rate of X-linked (Bruton) Agammaglobulinemia?
Which cell induces class switching in the B cell?
Name 2 advanced tests for phagocyte presence/function.
1. assays for phagocytosis, chemotaxis 2. genetics
What is a secondary immunodeficiency?
a process outside of the immune system has caused the problem
What enzyme to SCID pts lack?
adenosine deaminase (ADA)
What is the syndrome called where pts have high IgM with low IgG and IgA?
X-linked hyperIgM syndrome
People with CVID are at increased risk for ____, ____, and ____.
lymphoma, enteropathy, autoimmunity
T-cell deficiencies are assoc. with intracellular pathogens, especially _____ and _____.
Candida albicans; Pnemocystis jirovecii
What is the defect in X-linked hyperIgM syndrome?
IgM-to-IgG switch mechanism
___ deficiencies are assoc. with high grade bacterial infections, such as Staph. aureus, H. influenzae, and Strep. pneumoniae.
What is the syndrome where the thymus develops abnormally, resulting in absent T cells and normal B cells?
Name 2 initial tests for phagocyte presence/function.
1. WBC count, diff, morph 2. NBT test, oxidative burst
B-cell deficiencies are assoc. with ____, such as Staph. aureus, H. influenzae, and Strep. pneumoniae.
high grade bacterial infections
How is AT inherited?
How is Wiskott-Aldrich syndrome inherited?
What role does adenosine play in cellular development?
it accumulates in all cells, but impairs lymphocyte development in most severe
What is the cause of DiGeorge Syndrome?
large (45 gene) deletion on chromosome 22
What is the most common disease of immunodeficiency, and what is the incidence rate?
Selective IgA deficiency; 1/500
Name 4 initial tests for B cell presence/function.
1. serum protein electrophoresis 2. quantitative IgG, A, M levels 3. specific Abs to prior immunizations 4. ABO isohemagglutinins
What is the life span of a pt with SCID?
Name 2 advanced tests for T cell presence/function.
1. CD3, CD4, CD8 counts 2. mitogen responses, MLR, cytokine measurements
Name an advanced test for complement presence/function.
1. individual complement component levels
AT patients have a very high rate of tumors because of?
defective DNA repair
What ligand does the Tfh cell interact with on the B cell?
What is the most common form of SCID in Navajo children and what is the incidence rate?
defect in V(D)J recombination; 50/100,000
What are the s/s/findings in SCID?
lymphopenia of both T and B cells; absent thymic shadows; small tonsils; serum immunoglobulins are low
Primary immunodeficiencies affect ____ cells.
T or B, or both,
What is common variable immunodeficiency (CVID)?
B cells are difficult to trigger specific antibody; serum IgG low
What is telangiectasia?
dilated abnormal blood vessels
What is Transient Hypogammaglobinemia of Infancy?
slow IgG build up in affected children