Immunology - Defects with IS (Ch 19)
Defects in the Immune System
I. Primary Immunodeficiencies
A. Defects in phagocytosis
1. numbers of phagocytes
2. reduction in phagocytic function
a. chronic granulomatous disease
autosomal or X linked recessive
b. Chediak-Higashi syndrome
c. defects in chemotaxis
d. defects in adherence
B. Defects in complement
1. impaired opsonization
2. impaired chemotaxis
C. Stem cell deficiencies
1. reticular dysgenesis = no lymphoid or myeloid cells
2. severe combined immunodef. = no lymphoid cells
a. adenosine deaminase deficiency
b. ADA gene therapy
3. DiGeorge syndrome - Tcells
4. agammaglobulinemia - Bcells
5. def. with specific Ab (IgA, IgM, IgG)
D. Immunodef. mouse strains
II. Secondary immunodef.
1. malnutrition
2. exercise
3. trauma
4. age
5. toxins (environmental)
6. disease (AIDS)
III. AIDS
A. History
1. disease identified in 1981
2. HIV identified 1983
3. older names = HTLV III, LAV, ARV
4. current designations = HIV-1, HIV-2
5. HIV present in 1950s
B. Stats
1. worldwide
2. US
C. Characteristics of HIV
1. retrovirus
2. genome
3. genetic variability = Ag variation
4. host cell receptor = CD4
D. Tramsmission of HIV
1. sexual contact
2. blood and blood products
E. Diagnosis and symptoms
1. ELISA/Western blot
2. drop in levels of CD4+ cells
3. opportunistic infections
F. Treatment
1. AZT (3’-azido-2’,3’-dideoxythymidine), ddI
(2’,3’-dideoxyinosine), 3TC
(2’-deoxy-3’-thiacytidine)
2. triple cocktail (protease inhibitors and anti-viral drugs)
(protease inhibitors = saquinavir, ritonavir, indinavir)
G. Chemokine receptors
1. CCR5
2. CXCR4