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Immunology 5 - Immunodeficiency (ii) (Complement deficiency (assessing…
Immunology 5 - Immunodeficiency (ii)
Assessing Humoral Immunity
clinical Hx (look @ big picture, not each single infection)
measure Igs (subclasses + complement activity)
serum protein electrophoresis
quantitative + qualitative abnormalities (that would offer no protein)
functional assessment (NB as normal levels could be present, but no specific response)
natural or vaccine-induced (test vaccine = pneumovax)
check B cell nos
tonsils (90% B cells)
blood
BM
B cell prolif assay (not routine)
Neutrophil dysfunction
primary
genetic
rare
cause endothelial adhesion defects
chronic granulomatous disease
management
antibiotic (cotrimoxazole)
antifungal (itraconazole)
may have to prescribe a steroid if there's lots on inflamm
currently clinical trials for gene tx
leucocyte adhesion defect
hyper-IgE syndrome
only cure = haemopoetic stem cell transplant
good if Dx young + has HLA identical sibling
reduces mortality risk
if no sib parents are a 50% match
transplant mortality = 10-20%
higher when older
assess benefits vs risks
secondary
more common
drugs (e.g. steroids) affect migration or impair phagocytic activity
DM (neutrophil mobility impaired in a hyperglycaemic environment)
Fe deficiency (impairs bacterial killing)
assessing neutrophil function
Hx
check blood sugar, Ig levels, neutrophil morphology
resp burst (assesses their ability to make ROS)
check adhesion molecule expression
Complement deficiency
genetic: low levels/dysfunction
acquired: autoantibodies
classical pathway deficiency
C1/2/4
immune complex disease
alternative pathway deficiency
Factor D + P
susceptible to pyogenic Neisseria
common pathway deficiency
C3b
ICD + Nesseria
C5-9
Neisseria
assessing complement function: do 2 tests 1st
CH100
tests C1,2,4,3,5-9
AP100
tests FD,FP,C3,C5-9
if both +ve: common deficiency
then based on these findings find the individual protein
remember protein may be present @ normal levels but mutated (non-functional)
no cure
manage with vaccine, prophylactic antibiotics + patient education
General rules for managing immunodeficient patients
carefully chose antibiotic, its dose + duration (consider co-infection + opportunistic pathogens)
avoid live vaccines
explain genetic implications (family planning)
explain impact of travel + career (no microbiology/infectious disease!)
advise to wear medical alert jewellery