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Pharm immuno19 immunodeficiency

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  • Lymphocyte maturation pathways are described in more detail in Chapter 4.
  • Branchial pouches pharyngeal pouches paired evaginations of embryonic pharyngeal endoderm, between the branchial arches, extending toward the corresponding ectodermally lined branchial grooves; during development they evolve into epithelial tissues and organs, such as thymus and thyroid glands . Syn: branchial pouches, endodermal pouches Hypoplasia (h º” p ½ -pl ³“ zh … , -zh ¶ - … ) n. Incomplete or arrested development of an organ or a part
  • Examples showing the sites where immune responses may be blocked are illustrated in A, and the features of some of these disorders are summarized in B
  • Transcript

    • 1. Pharmacy-Immunology 19 Primary & Acquired Immunodeficiency Saber Hussein
    • 2. Objectives
      • Know the types of primary (congenital) immunodeficiency that result of:
        • Defects in B and T lymphocytes maturation
        • Defects in lymphocytes activation and function
        • Defects in innate immunity
        • Lymphocyte abnormalities associated with other diseases
      • Know the acquired (secondary) immunodeficiency syndrome (AIDS) in regard of:
        • Structure and biology of HIV
        • Clinical features of HIV infection and pathogenesis of AIDS
        • AIDS therapeutic and vaccination strategies
    • 3. Features of immunodeficiency diseases
      • Diagnostic features and clinical manifestations of immune deficiencies affecting components of the immune system
      • Different diseases, and even different patients with the same disease, may show considerable variation
      Fig12-1 (Extracellular bacterial infections)
    • 4. Congenital immunodeficiencies
      • Primary Immunodeficiencies are congenital diseases caused by genetic defects that inhibit maturation of B or T cells or interfere with functions of immune components
      • Prevalence : 1 in 500 American and European
      • Hallmark of immunodeficiencies’ consequences:
        • Infections that could be mild or severe, start in early childhood or in adulthood
    • 5. Congenital immunodeficiencies caused by defects in lymphocyte maturation
      • Autosomal SCID (severe combined immunodeficiency) caused by:
        • ADA (adenosine deaminase) & PNP (purine nucleoside phosphorylase) deficiency prevent maturation of:
          • Stem cell  pro-B cell
          • Pro-B cell  pre-B cell
          • Stem cell  pro-T cell
          • Pro-T cell  pre-T cell
        • RAG (recombination activating gene) deficiency prevents maturation of:
          • Pro-B cell  pre-B cell
          • Pro-T cell  pre-T cell
      • X-linked SCID:
        • Signaling IL-2Rg chain (  c is common in IL receptors of IL-2, IL-4, IL-7 , IL-9, IL-15) deficiency interferes with
          • Pro-T cell  pre-T cell [see Fig12-2, next slide]
    • 6. Fig12-2:Congenital immunodeficiencies Fig12-2:Congenital immunodeficiencies
    • 7. Features of congenital immunodeficiencies caused by defects in lymphocyte maturation Fig12-3
    • 8. Features of congenital immunodeficiencies caused by defects in lymphocyte maturation Fig12-3
    • 9. Congenital immune-deficiencies associated with defects in lymphocyte activation and effector functions
      • Congenital immunodeficiencies may be caused by genetic defects in the expression of molecules required for
        • Ag presentation to T cells ,
        • T or B lymphocyte antigen receptor signaling ,
        • helper T cell activation of B cells and macrophages , and
        • differentiation of antibody-producing B cells .
      Fig12-4 : Sites where immune responses may be blocked
    • 10. Congenital immunodeficiencies associated with defects in lymphocyte activation and effector functions Fig12-4: Features of some of resulting deficiency disorders
    • 11. Congenital immunodeficiencies caused by defects in innate immunity Fig 12-5
    • 12. Congenital immunodeficiencies caused by defects in innate immunity Fig 12-5
    • 13. Acquired (secondary) immunodeficiency diseases Fig 12-6
    • 14. HIV life cycle Fig 12-8 HIV life cycle
    • 15. The pathogenesis of HIV-AIDS
      • The stages of HIV disease correlate with a progressive spread of HIV from the initial site of infection to lymphoid tissues throughout the body.
      • The immune response of the host temporarily controls acute infection but does not prevent establishment of chronic infection of cells in lymphoid tissues.
      12-9
    • 16. The pathogenesis of HIV-AIDS
      • The host’s immune response temporarily controls acute infection
      • But establishment of chronic infection of cells in lymphoid tissues succeeds
      • Cytokines produced in response to HIV and other microbes serve to enhance HIV production and progression to AIDS
      12-9
    • 17. The clinical course of HIV disease
      • Blood-borne HIV virus (plasma viremia) is detected early after infection
      • It may be accompanied by systemic symptoms typical of acute HIV syndrome
      • The virus spreads to lymphoid organs , but
      • plasma viremia falls to very low levels (only detectable by sensitive reverse transcriptase polymerase chain reaction (rtPCR) assays) & stays this way for many years
      • CD4 + T cell counts steadily decline during this clinical latency period , because of
        • active viral replication and
        • T cell destruction in lymphoid tissues
      • As the level of CD4 + T cells falls , there is increasing risk of infection and other clinical components of AIDS
      • [See next slide, Fig 12-10 ]
    • 18. The clinical course of HIV disease Fig 12-10