Pharm immuno19 immunodeficiency
Upcoming SlideShare
Loading in...5
×
 

Pharm immuno19 immunodeficiency

on

  • 364 views

 

Statistics

Views

Total Views
364
Views on SlideShare
364
Embed Views
0

Actions

Likes
0
Downloads
2
Comments
0

0 Embeds 0

No embeds

Accessibility

Upload Details

Uploaded via as Microsoft PowerPoint

Usage Rights

© All Rights Reserved

Report content

Flagged as inappropriate Flag as inappropriate
Flag as inappropriate

Select your reason for flagging this presentation as inappropriate.

Cancel
  • Full Name Full Name Comment goes here.
    Are you sure you want to
    Your message goes here
    Processing…
Post Comment
Edit your comment
  • 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

Pharm immuno19 immunodeficiency Pharm immuno19 immunodeficiency Presentation Transcript

  • Pharmacy-Immunology 19 Primary & Acquired Immunodeficiency Saber Hussein
  • 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
  • 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)
  • 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
  • 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]
  • Fig12-2:Congenital immunodeficiencies Fig12-2:Congenital immunodeficiencies
  • Features of congenital immunodeficiencies caused by defects in lymphocyte maturation Fig12-3
  • Features of congenital immunodeficiencies caused by defects in lymphocyte maturation Fig12-3
  • 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
  • Congenital immunodeficiencies associated with defects in lymphocyte activation and effector functions Fig12-4: Features of some of resulting deficiency disorders
  • Congenital immunodeficiencies caused by defects in innate immunity Fig 12-5
  • Congenital immunodeficiencies caused by defects in innate immunity Fig 12-5
  • Acquired (secondary) immunodeficiency diseases Fig 12-6
  • HIV life cycle Fig 12-8 HIV life cycle
  • 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
  • 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
  • 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 ]
  • The clinical course of HIV disease Fig 12-10