assessment 7 Flashcards
Dx that is a result of genetic deficiency of FoxP3 in Tregs autoimmunity :
IPEX
Type of virus – influenza?
Negative sense RNA virus
Antigenic shift causes huge change in this influenza protein:
hemagluttination
Insect born protozoa that replicate in EC tissue spaces:
trypanosomes
Trypanosomes coated with single type of glycoprotein:
VSG
Trypanosome evasion of host immune system?
Programmed re-arrangement of DNA: cassette system to express only 1 of 1000 different VSG (variant specific glycoprotein)
State in which no viral proteins produced (no disease caused, no CTL’s active) –
Latency
Herpes simplex virus occupies what cells?
Nerve
Why are neurons susceptible to viral infections?
Low # MHC I to decrease unnecessary killing by CTL’s
Mononucleosis is caused by?
Epstein-Barr Virus
Epstein-Barr infects what cells?
Causes them to proliferate until they are killed by CTL’s: B Cells
Pathogens most likely to subvert host defenses?
Viruses
Mycobacterium TB resistance to immune system?
Prevents fusions of lysosome and phagosome
Listeria evasion of immune system?
Escapes phagosome until macrophage killed by CTLs
Toxoplasma gondii evasion of the immune system?
Makes its own vesicles inside phagosome
What produces toxic shock syndrome toxin 1?
Staphylococcus
What does a super antigen bind?
Vb region of TCR and MHC II
Superantigen activates… % T cells?
2%-20%
Superantigen causes…
Massive release of cytokines
Phase of Ag specific Antibody present and detectable in the serum
Seroconversion
Lentivirus? Example?
slow, HIV
HIV: profound decrease in?
CD4 cells
HIV binds with high affinity to_____ and must interact with_____ to gain entry into the cell.
CD4 and chemokine receptor
Antigenic variation in HIV is due to _____?
Error prone reverse transcriptase
Asplenia increases susceptibility to?
Septic infections (encapsulated bacteria)
Neutropenia is a deficiency in _____? must be less than ____ cell/ul?
Granulocytes, <500 cells/ul
Severe congenital neutropenia called____? defect?
Kostman syndrome, G-CSF or its receptor
Autosomal dominant type of neutropenia that occurs every 2-4 weeks, defect in ELA-2?
Cyclic neutropenia
Absence/non functional NK and NKT cells?
Absolute NKD
Absence of NK cells/ function?
Classical NKD
Loss of NK cell fuction?
Functional NKD
Most toll signals through what Trx factor?
NF-KB
X linked hypohydrotic ectodermal dysplasia immunodeficiency= defect in what protein?
NEMO
Usual results of deficiencies in complements?
EC bacteria
Deficiencies in complement regulatory proteins can cause what 2 problems?
- Encapsulated bacterial susceptibility due to depletion of Cs
- Destruction of RBCs
Mannose Binding Lectin Deficiency: increases … meningitis?
Neisseria
Paroxymal Nocturnal Hemoglobinuria caused by? Complement induced intravascular hemolytic anemia due to
def … and …
DAF and CD59 (Protects host cells)
X-linked agammaglobulinemia caused by defect in? Vulnerable to? ……; leads to lack of signal transduction in B
cells; 2 pathogens?
Bruton’s tyrosine kinase leads to lack of signal transduction in B cells; EC bacteria and cannot neutralize viruses
Surrogate light chain that pairs with mu heavy chain? (deficiency causes lack of pre-B receptors)
Gamma 5
2 mutations that can cause X-linked hyper IgM syndrome:
1- defect in CD40L (no 2nd activation) 2-deficiency in activation induced cytidine deaminase (cannot isotype
switch or somatic hypermutate)
IgA deficiency leads to increased susceptibility to?
Parasites
Selective IgG deficiency – most common in kids? In adults? Kids: … and adults ….
Kids: G2 and adults G3
Group of 150 primary immunodeficiencies: reduced levels of antibodies; most common primary immunodeficiency disorder: (dx in 20s or 30s):
common variable immunodeficiency
Classical bare lymphocyte syndrome is a deficiency in …?
MHC II; no positive selection of CD4 cells in thymus
2 defects to cause defect in CD8 function?
1-TAP deficiency 2-CD8 alpha chain defect
Defect in cytoskeletal reorganization that is needed for T cells to deliver cytokines and other signals to B cells and macrophages – …… (SCID – T cells cannot talk to B cells)
Wiskott-Aldrich syndrome
Adenosine deaminase deficiency (ADA) or purine nucleotide phosphorylase def results in?
Accumulation of toxic nucleotide catabolites that kill developing B and T cells
CD4 defects can result in ______?
CD4 defects can result in… SCID (severe combined immunodeficiency – CD4 are important for cell-mediated and humoral response)
Common gamma chain def, Jak3, and CD3 def result in?
defects in T cell function causing SCID
Mis-sense mutation that partially inactivates RAG causing absence of B cells and oligoclonal autoreactive T-cells
– (shares a phenotype with common gamma chain def)
Omenn Syndrome
DiGeorge results in small deletion in what chromosome resulting in what?
Chromosome 22, no thymus
APECED, results in a myriad of autoimmune diseases due to a deficiency in what?
AIRE
IPEX , onset 1st year of life: 3 symptoms?
Watery diarrhea, eczematous dermatitis, endocrinopathy
Role of Factor P (properdin) – enhances…
enhances the alternative complement pathway (prevents destruction of C3b)
Genetic dx characterized by lymphadenopathy and splenomegaly: immune cells fail to … following immune
response –> overpopulation of secondary lymphoid tissue:
ALPS (autoimmune lymphoproliferative syndrome)
Delivery of initial smallpox vaccine –
variolation
Virus used to vaccinate against smallpox (cow pox)
vaccinia (vacus = cow)
Only part of pathogen is used as immunogen =
subunit vaccine
Inactivated toxin
toxoid
Most widely used live bacterial vaccine that is not used in the US is used against? And called?
BCG Bacille-Calmette –Guerin; Mycobacterium tuberculosis
What does DTP vaccine consist of and what does it target?
Diphtheria & tetanus toxoids combined with killed Bordetalla pertussis (whopping cough)
2 killed virus vaccines?
Salk polio, influenza
Most anti-virals are what?
Live attenuated vaccines
Name 4 live-attenuted viral vaccines:
measles, mumps, Sabin polio, yellow fever
Substance that enhances the immunogenicity of the antigen –
Adjuvant
Adjuvants can turn a … protein antigen into a … antigen which is more readily taken up by APCs
soluble > particulate
What is added as a source of PAMPs and to increase inflammation around vaccine?
Mycobacterial cell wall components
