Immunodeficiency Flashcards
What are the four limbs of the immune system? (C,H,C,P)
Cellular Immunity
Humoral Immunity
Complement
Phagocytes
What is the distribution of Primary Immunodeficiencies?
Humoral Immunity Phagocyte Cellular Complement Combined
What is a disease brought on by problems with phagocytes?
Chronic Granulomatous DIsease (CGD)
What occurs in CGD?
It is NOT loss of phagocytes, just loss of SPECIFIC function
What is a disease brought on by a COMBINED IMMUNODEFICIENCY?
X-linked SCID
What is the reason for X-linked SCID?
There is a defect in IL2 gamma chain (signals transduce through this chain) - FUNCTIONS ARE LOST
Treatment?
They are usually prone to EVERY TYPE of infection
- so needs to be treated aggressively
- need transplant
What is a disease brought on by HUMORAL IMMUNODEFICIENCY?
Bronchiectasis - Damage to airways and causes them to widen and become flabby and scarred
How to determine person has this?
History and X-ray findings
B-cell enumeration
Quantitative Igs
Can you give vaccines to these people?
Yes, but NOT live vaccines
**What is a disease brought on by HUMORAL IMMUNODEFICIENCY?
X-linked Agammaglobulinemia
What is X-linked Agammaglobulinemia
- Absence of BCs due to mutations in BTK
- CD40 ligand deficiency
- ->plenty of IgM but not IgG
- ->no germinal centers
**What is a disease brought on by CELLULAR IMMUNODEFICIENCY?
DiGeorge Syndrom
-Lack of thymus
Often given Ab – w/o T-cells they don’t help B-cells and cant make Ab
**What is a disease brought on by COMPLEMENT IMMUNODEFICIENCY?
Meningitis
-deficiency in C8
Can they receive vaccines?
This child load up with vaccines
Can receive live vaccines (T and B cells are normal)
