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30 Cards in this Set
- Front
- Back
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Name 2 phagocytic cell dysfunctions and what is defective
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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. |
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3 "sign-posts of immune status" of antibody-mediated immunity
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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 |
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The presence of Staphylococcal protein A would be a sign of what type of immunodeficiency?
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antibody-mediated immunity
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2 "sign-posts of immune status" of cell-mediated immunity
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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 |
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The presence of Phytohemmagglutinin [PHA] would be a sign of what type of immunodeficiency?
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cell-mediated immunity
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What is SCID? what does it affect? What are the two types?
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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. |
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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 |
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50% result the autosomal deficiency of SCID is due to what defective enzyme?
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adenine deaminase [ADA] or purine nucleoside phosphorylase [PNP]
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What is the Bare-leukocyte syndrome?
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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. |
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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.
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What will a RAG deficiency result in?
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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 |
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What is x-linked SCID caused by?
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caused by defective expression of the common gamma chain associated with receptors for several important cytokines (IL-2, 4, 7, 9, 15).
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What is the importance of a defective IL-7?
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defective IL-7 receptor may be of exceptional significance, due to its importance with regard to the development of early lymphocyte progenitors.
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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 |
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What is X-linked congenital agammaglobulinemia and whom is it most common in?
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Results from a defective tyrosine kinase (btk) necessary for appropriate V to DJ joining. Stuck in pre-B cell stage. Present in young boys.
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What is Common variable immunodeficiency (CVI) and whom is it most common in?
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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. |
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Most common of any immunodeficiency is ....
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selective IgA deficiency (1 in 400 individuals)—mostly asymptomatic.
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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. |
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What is the result of Activation-Inducted Cytidine deaminase [AID] deficiency?
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results in B cell w/ defective abilities to class switch and affinity maturation
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Name 3 deficiencies in cell-mediated immunity
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Digeorge’s Syndrome
Mucocutaneous Candidiasis AIDS |
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What is Digeorge’s Syndrome from?
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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 |
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What is Mucocutaneous Candidiasis from?
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Selective dysfunction of the DTH arm of the T cell compartment
-other T cell functions appear normal. Can become systemic and often fatal. |
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Immune impairment of HIV is a result of decreased numbers of ..... [3 cells]
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CD4 positive inducer T cells, dendritic cells and macrophages
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What does HIV use as a receptor for entry into the cell? What chemokines act as co-receptors?
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uses CD4 as a receptor for entry into the cell
chemokine receptors: CCR5 on T cells and Macs, and CXCR on ONLY T cells |
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What is Kaposi sarcoma?
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a carncer that HIV patients have a increased incidence of
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Name 3 tools to monitor the course of an HIV infection
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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 |
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What is a normal CD4 count?
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1200 /microliters
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What are the CD4 number for HIV asymptomatic individuals?
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800 /microliters
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What are CD4 numbers as opportunistic infections first begin to emerge?
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<500 /microliters
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What is the CD4 count for full blown AIDS?
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<200/microliters
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