Primary (usually hereditary) and secondary (acquired)
What are the two types of immunodeficiencies?
Deficiency in T cells and may affect B cells and/or NK cells, deficient cellular and humoral immunity, myeloid and erythroid cells are normal, recurrent infections
What are some common characteristics of SCID?
This is caused by a mutation to the γ-chain cytokine receptor
Which cytokine receptors contain the γ-chain?
Adenosine deaminase (ADA) deficiency
This leads to no DNA synthesis and thus no clonal expansion of lymphocytes in SCID
Bare Lymphocyte syndrome
This is the lack of MHC II expression which causes CD4 T-cells to not be activated
Bruton's tyrosine kinase
component of signal transducing chain of B cell receptor that is absent in X-linked agammaglobulinemia
Lack of Btk, few B-cells, few lymphoid follicles, little antibody produced, recurrent bacterial infections (fungi and virus less affected)
What are some characteristics of X-linked agammaglobulinemia?
Recurrent infections, low levels of IgA, IgG, and IgE, normal IgM and IgD, defect in CD40L, Normal B cell numbers and normal TI responses
What are some characteristics of X-linked hyper-IgM syndrome?
Required for class switching if immunoglobulins
Why is CD40L important?
Phagocytic cell defects characterized by frequent bacterial infections, affects early myeloid precursor cells, and often due to a defect in GM-CSF
Chronic Granulomatous disease (CGD)
Phagocytic cell defect characterized by phagocytes that produce no ROIs and thus cannot kill pathogens
What does the chronic activation of macrophages by T cells lead to in CGD?
What is the main pathogen associated with Chronic granulomatous disease?
Aggressive antibiotics and IFN-γ to stimulate ROIs
What therapy is used to combat Chronic granulomatous disease?
CGD which is caused by defect in p91phox subunit
CGD which is caused by defect in p67phox subunit
Disease in which Neutrophils, monocytes, and lymphocytes have "giant" lysosomes and thus phagocytic functions are defective
This is caused by defective melanosomes
Leukocyte adhesion deficiency
Disease caused by the Lack of β-chain common to LFA-1, CR3, CR4 in numerous types of leucocytes and functions
Increase in immune complex diseases and failure to generate C3b and thus cannot clear immune complexes
What are the effects of early classical complement defects for C1, C4, and C2?
Factor H or factor I deficiency
This causes C3b to uncontrollably combine with factor B and form more C3 convertase and consume all C3
Mutation of C3 gene
this defect causes a lack of opsonization and lack of chemotaxis
Lack of MAC (C5, 6, 7, or 8 deficiency)
This causes increased susceptibility to Neisseria gonorrhea and N. Meningitidas
Serum antibody levels, response to immunizations, enumerate B-cells
What is used to recognize humoral immunodeficiencies?
Measure T-cell numbers, response to skin tests, and measure cytokine production of cultured lymphocytes
What is used to recognize cellular immunodeficiencies?
What type of virus is HIV?
the transmission of disease from an animal to humans
What is used by HIV to transcribe viral RNA into DNA?
Reverse transcriptase, protease, integrase
What are the 3 enzymes important to HIV survival?
Which part of the HIV genome encodes for capsid?
Which part of the HIV genome encodes for the 3 enzymes?
Which receptor does HIV attach to?
CCR5 and CXCR4
What are the coreceptors on CD4 cells that bind HIV?
Viral envelope gp120
Which part of HIV binds to CD4?
This mediates the viral DNA integration into human nuclear DNA
T-cell or macrophage stimulation during immune response
What leads to HIV proviral transcription?
What cleaves the viral precursor proteins into mature proteins?
Half life of 44 months
Why is it hard to clear out the reservoir of HIV infection?
Lymphoid tissue, peripheral blood dendritic cells and monocytes, resting CD4 lymphocytes, microglial cells, reproductive tract, GI tract
What are the common reservoirs of HIV infection?
HIV RNA copies per mL plasma
What is the HIV viral load?
Pneumocystis carinii pneumonia, cryptococcosis, toxoplasmosis, cytomegalovirus disease, mycobacterium avium complex disease
What are common opportunistic infections of AIDS?
Nucleoside Reversie transcriptase inhibitors, Non-nucleoside reverse transcriptase inhibitors, protease inhibitors, fusion inhibitors, CCR5 inhibitors, integrase inhibitors
What are the5 classes of antiretroviral drugs?
Resistance, adverse effects, drug administration
What are the problems with current drugs?
Diversity, evade adaptive immune response, latent reservoirs
Why is it difficult to develop a successful HIV vaccine?
What is the main strain of HIV-1 in North America?
Vaccine that consists of arts of HIV and produce a weak immune response
Recombinant vector vaccines
Vaccine that uses non-HIV viruses to act as vector for HIV genes and uses HIV genes to produce proteins that initiate an immune response
Contains HIV genes independent of a vector and this DNA is taken up by the cell and produces proteins that initiate immune response
HDAC inhibitors, IL-7, HMBA, Paustratin
What are therapeutic agents for disruption of latent proviral HIV infection and thus activate transcription of HIV virus?