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30 Cards in this Set

  • Front
  • Back
Name 2 phagocytic cell dysfunctions and what is defective
Leukocyte adhesion deficiency: defective integrin affecting the ability of leukocytes to move from the blood stream to the site of infection

Chronic granulomatous disease: defective superoxide production affecting the ability of phagocytes to destroy ingested bacteria.
3 "sign-posts of immune status" of antibody-mediated immunity
1)Serum antibody levels--circulating immunoglobulins
2)Check B cell levels in the circulation--Surface Ig positive cells
3)Check functional status of the B cells--checking to make sure they are working right--mitogen: polyclonal activator
The presence of Staphylococcal protein A would be a sign of what type of immunodeficiency?
antibody-mediated immunity
2 "sign-posts of immune status" of cell-mediated immunity
1)Check T cell levels in circulation--determined by CD2+ (adhesion molecules) cells. Can also look at specific type of T-cells present—circulating T cells—CD3 (all mature T cells), CD4 and CD8.
2)Check functional status of T cells--evaluate T cell responses against antigens associated with microorganisms commonly found in our environment
The presence of Phytohemmagglutinin [PHA] would be a sign of what type of immunodeficiency?
cell-mediated immunity
What is SCID? what does it affect? What are the two types?
Severe Combined Immunodeficiency Disease --
Caused by defective differentiation of early progenitor cells of the immune system—may involve multi-potential stem cell. Either autosomal or sex-linked.
What is the result of SCID?
How can you treat it?
Results in a complete lack of T cells and B cells—some forms have B cells present, but they are not functional due to lack of T cells required for activation.

Death usually occurs within first year after birth.

Treatment: antibiotics, gammaglobin administration, transplantation of bone marrow, stem cells or thymus
50% result the autosomal deficiency of SCID is due to what defective enzyme?
adenine deaminase [ADA] or purine nucleoside phosphorylase [PNP]
What is the Bare-leukocyte syndrome?
autosomal SCID:

family of disorders that involves deficient expression or function of either Class I or Class II HLA molecules

result: impaired development or activation of subsets of T cells.
The Class I deficiency in Bare-leukocyte syndrome is due to a mutation in what?
what is the result?
TAP genes, --resulting in impaired antigen presentation to CD8+ cells.
What will a RAG deficiency result in?
autosomal SCID:

impaired expression of recombination activation genes (RAG1/RAG2) necessary for the proper genetic rearrangement required for production of the antigen receptors on B and T cells
What is x-linked SCID caused by?
caused by defective expression of the common gamma chain associated with receptors for several important cytokines (IL-2, 4, 7, 9, 15).
What is the importance of a defective IL-7?
defective IL-7 receptor may be of exceptional significance, due to its importance with regard to the development of early lymphocyte progenitors.
What are agammaglobulinemias?

What is the result?
Deficiencies in antibody production

Involves maturation or regulatory defects in which B cells are either physically missing or are present but dysfunctional. May be generalized or restricted. Defects in antibody production may also be the indirect result of faulty regulation by helper T cells
What is X-linked congenital agammaglobulinemia and whom is it most common in?
Results from a defective tyrosine kinase (btk) necessary for appropriate V to DJ joining. Stuck in pre-B cell stage. Present in young boys.
What is Common variable immunodeficiency (CVI) and whom is it most common in?
Similar to x-linked agamma in that immunoglobulin levels are decreased.
-affects an older patient population, B cells present (but no plasma cells), frequencies of affected males and females equal due to autosomal inheritance. Mechanisms unknown.
Most common of any immunodeficiency is ....
selective IgA deficiency (1 in 400 individuals)—mostly asymptomatic.
What is Hyper-IgM caused by?
what do you get an accumulation of?
a defective in CD40 ligand on activated T cells.

therefore get accumulation of B cells unable to switch from IgM to IgG or other isotypes.
What is the result of Activation-Inducted Cytidine deaminase [AID] deficiency?
results in B cell w/ defective abilities to class switch and affinity maturation
Name 3 deficiencies in cell-mediated immunity
Digeorge’s Syndrome
Mucocutaneous Candidiasis
What is Digeorge’s Syndrome from?
Thymic aplasia due to grossly abnormal embryonic development during the 12th week of gestation.

-see striking set of facial features—useful for diagnosis. Pre-T cells are present. B cells are present but demonstrate variable function due to improper regulation in the absence of functional T cells
What is Mucocutaneous Candidiasis from?
Selective dysfunction of the DTH arm of the T cell compartment

-other T cell functions appear normal. Can become systemic and often fatal.
Immune impairment of HIV is a result of decreased numbers of ..... [3 cells]
CD4 positive inducer T cells, dendritic cells and macrophages
What does HIV use as a receptor for entry into the cell? What chemokines act as co-receptors?
uses CD4 as a receptor for entry into the cell

chemokine receptors: CCR5 on T cells and Macs, and CXCR on ONLY T cells
What is Kaposi sarcoma?
a carncer that HIV patients have a increased incidence of
Name 3 tools to monitor the course of an HIV infection
1. ELISA Assay to secreen for HIV serepositivity
2. PCR to test for presence of HIV genome
4. use immunoflourescense w/ monoclonal antibodies to test for circulating CD4+ cells
What is a normal CD4 count?
1200 /microliters
What are the CD4 number for HIV asymptomatic individuals?
800 /microliters
What are CD4 numbers as opportunistic infections first begin to emerge?
<500 /microliters
What is the CD4 count for full blown AIDS?