Principles of Immunology Immunodeficiency 4/20/06 - PowerPoint PPT Presentation

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Principles of Immunology Immunodeficiency 4/20/06

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Wiskott Aldrich Syndrome. X linked disorder. Affects platelet numbers/function ... DiGeorge Syndrome. Poorly developed or functioning thymus ... – PowerPoint PPT presentation

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Title: Principles of Immunology Immunodeficiency 4/20/06


1
Principles of ImmunologyImmunodeficiency4/20/06
  • Wise people talk because they have something to
    say fools, because they have to say something
  • Plato

2
Word/Terms List
  • AIDS
  • SCID
  • Primary immunodeficiency
  • Secondary immunodeficiency

3
Immunodeficiency
  • Primary-usually genetic, congenital
  • Secondary-Acquired

4
Mechanisms of Immunodeficiency
  • Loss or reduction of
  • Cell type
  • Cell numbers
  • Cell function

5
Loss of Cell Function
  • Receptors
  • Cell signaling
  • Cytokine production
  • Ig production
  • Co stimulation impairment
  • Intracellular killing
  • Extravasation impairment

6
(No Transcript)
7
Primary Immunodeficiency
  • Myeloid lineage
  • Congenital agranulocytosis
  • Leukocyte-adhesion deficiency
  • Lymphoid lineage
  • Severe combined immunodeficiency (SCID)
  • B cells
  • Agammaglobulinemia
  • Hypogammaglobulinemia
  • Specific Ig Deficiencies
  • T cells
  • DiGeorge Syndrome
  • Wiskott Aldrich Syndrome

8
(No Transcript)
9
Secondary Immunodeficiency
  • Drug related
  • Disease related
  • Cancer
  • AIDS
  • HIV
  • T helper cell as target

10
SCID
  • Various genetic defects
  • No TCR or defective TCR
  • Defective cell signaling
  • Defective IL 2
  • Recurrent infections
  • Death at early age

11
Wiskott Aldrich Syndrome
  • X linked disorder
  • Affects platelet numbers/function
  • Affects T cell function
  • Cytoskeleton of lymphocytes affected
  • Lower amounts of IgM
  • Increased susceptibility to certain bacterial
    infections

12
Brutons Agammaglobulinemia
  • Low levels of IgG
  • B cell signal transduction affected
  • Defective BCR
  • Recurrent bacterial diseases starting at end of
    first year of life
  • Short life span

13
DiGeorge Syndrome
  • Poorly developed or functioning thymus
  • Associated with other developmental conditions
  • Depression of T cell numbers
  • Absence of T cell response
  • Humoral response to T independent antigens only

14
(No Transcript)
15
Secondary Immunodeficiency
  • Drugs
  • Irradiation
  • Cancer
  • AIDS

16
AIDS
  • HIV as agent
  • T helper as target cell
  • Attacked by own CD8 cells
  • Susceptible to opportunistic infections
  • Kaposis sarcoma
  • CMV
  • Candida
  • Pneumocystis carinii
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