Principles of Immunology Immunodeficiency 4/20/06

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Principles of ImmunologyImmunodeficiency4/20/06

• Wise people talk because they have something to
  say fools, because they have to say something
• Plato

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Word/Terms List

• AIDS
• SCID
• Primary immunodeficiency
• Secondary immunodeficiency

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Immunodeficiency

• Primary-usually genetic, congenital
• Secondary-Acquired

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Mechanisms of Immunodeficiency

• Loss or reduction of
• Cell type
• Cell numbers
• Cell function

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Loss of Cell Function

• Receptors
• Cell signaling
• Cytokine production
• Ig production
• Co stimulation impairment
• Intracellular killing
• Extravasation impairment

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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

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Secondary Immunodeficiency

• Drug related
• Disease related
• Cancer
• AIDS
• HIV
• T helper cell as target

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SCID

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

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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

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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

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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

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Secondary Immunodeficiency

• Drugs
• Irradiation
• Cancer
• AIDS

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AIDS

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