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Principles of Immunology Immunodeficiency 4/20/06

Principles of Immunology Immunodeficiency 4/20/06. ”Wise people talk because they have something to say; fools, because they have to say something” Plato. Word/Terms List. AIDS SCID Primary immunodeficiency Secondary immunodeficiency. Immunodeficiency. Primary- usually genetic, congenital

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Principles of Immunology Immunodeficiency 4/20/06

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  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. 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

  7. Secondary Immunodeficiency • Drug related • Disease related • Cancer • AIDS • HIV • T helper cell as target

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

  9. 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

  10. Bruton’s 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

  11. 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

  12. Secondary Immunodeficiency • Drugs • Irradiation • Cancer • AIDS

  13. AIDS • HIV as agent • T helper as target cell • Attacked by own CD8 cells • Susceptible to opportunistic infections • Kaposi’s sarcoma • CMV • Candida • Pneumocystis carinii

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