Lecture 21

1.
What are primary immune deficiencies?
Genetic mutation
2.
What are secondary immune deficiencies?
Even that damages immune system
3.
How many genetic mutations result in a primary immune deficiency?
180
4.
How common are primary immune deficiencies?
1 in 5000
5.
What happens with defects in phagocyte response?
Recurrent infections
6.
What are the types of defects in phagocyte response?
"Production of phagocytes, adhesion, activation, killing of microbes"
7.
What mutation affects leukocyte adhesion?
Defect in LFA-1 gene
8.
What are the symptoms of leukocyte adhesion deficiencies?
"White cell trafficking problems, compromised wound healing, absence of pus (no neutrophils)"
9.
What is chronic granulomatous disease?
Phagocytes can't produce ROS
10.
What causes chronic granulomatous disease?
Mutation of NADPH oxidase
11.
What are the results of primary defficiencies in complement pathway?
"Increased susceptibility to bacteria requiring complement to remove eg. opsonisation, defects in C3 lead to increased susceptibility to Neisseria spp, defects in MAC, increased susceptibility to Neisseria"
12.
What is the result of primary immune deficiency of B cells?
"Defects in antibody production, recurrent infections with pyogenic bacteria, first occurs 7-9 months after birth"
13.
What is the result of primary immune deficiency of T cells?
"Functional defects of T cells, defects in T cell - APC cell interaction"
14.
What is SCID?
Severe combined immunodeficiency
15.
What is responsible for 25% SCIDs?
Deficiency in adenosine deaminase => increased ATP => Toxicity to B and T cells
16.
What is another version of SCIDs?
X-linked mutations in gamma chain of interleukin 2 receptors => T cells can't grow
17.
What are the symptoms of SCIDs?
"Diarrhoea, ear infections, fungal infections, oral/skin/gut infections, mortality by 2 years"
18.
What can cause secondary immundeficiency?
"Infection, aplastic anemia, drugs, chronic disease, age"
19.
What is the mechanism of HIV?
Virus invades CD4+ T cells
20.
What is the mechanism of aplastic anemia?
"Stem cells in bone marrow are destroyed => Decreased RBCs, WBCs, platelets"
21.
What are the symptoms of aplastic anemia?
"Fatigue, infection, bruises"
22.
What are the causes of aplastic anaemia?
Exposure to chemicals/radiation
23.
How do chemotherapy drugs cause immunodeficiency?
"Destroy rapidly proliferating cells => decreased cancer, decreased RBCs, decreased WBCs"
24.
How do immunodeficiency drugs affect immune system?
Reduce leukocyte function
25.
What are examples of latent virus reactivation?
"EBV, herpes virus, varicella"
26.
Where does the herpes virus lay dormant?
Trigeminal ganglion
27.
Where does varicella lay dormant?
Dorsal root
28.
What does varicella - zoster virus reactivate as?
Shingles
29.
What causes infective endocarditis?
No blood supply to heart valve tissue => no immune access
30.
What other sites can be affected?
"Stents, joint replacement, breast, cochlear implants"