MedEd Flashcards
Soluble components of the innate immune system
- complement
- acute-phase proteins
- cytokines and chemokines
Cells of the innate IS
- PMN cells (neutrophils, eosinophils, basophils)
- monocytes and macrophages
- NK cells
- dendritic cells
What are the 3 ways complement is activated?
- classical pathway (C1, C2, C4) - Ag-Ab complexes
- mannose-binding lectin (MBL) pathways
- alternative pathway
Name 2 conditions causing failure to produce neutrophils and how you treat them
- Kostmann syndrome (AR)
- cyclic neutropenia (AD)
-> granulocyte colony-stimulating factor (G-CSF) as treatment
Mutation in kostmann syndrome
HAX-1 protein (AR)
Mutation in cyclic neutropenia
ELA-2 (AD)
Treatment for leukocyte adhesion deficiency
HSCT
What is chronic granulomatous disease?
- defect of oxidattive killing
- deficiency of NADPH oxidase complex (which is responsible for making ROS), -> impaired killing
What is chronic granulomatous disease?
- defect of oxidattive killing
- deficiency of NADPH oxidase complex (which is responsible for making ROS), -> impaired killing
Sx of chronic granulomatous disease
pus
granuloma formation
lymphadenopathy
hepatosplenomegaly
Mx of Chronic granulomatous disease
IFN-gamma
Catalase Positive organisms
Pseudomonas aeruginos Listeria A Aspergillus Candida E. coli Staph aureus Serratia
-> common infections in chronic granumlomatous disease
Tests for chronic granulomattous disease
- nitroblue tetrazolium test (changes from yellow to blue with hydrogen peroxide) Positive test is normal
- dihydrorhodamine flow cytometry test (DHR is oxidised to fluorescent rhodamine with hydrogen peroxide)
- > both indicate hydrogen peroxide presence (no change in colour indicates chronic granulomatous disease)
Cytokine deficiency
- IL-12 and IFN-gamma commonly affected (or IL-12R and IFN-gamma R)
cytokine deficiency - what infections are common?
Intracellular infections
- mycobacterial
- salmonella