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Immunology 5 - Immunodeficiency (iii) (T cell/combined IDs (secondary (HIV…
Immunology 5 - Immunodeficiency (iii)
T cell/combined IDs
primary
SCID
ADA deficiency
toxic intracellular dATP buildup - prevents DNA synthesis, so mitotically active lymphocytes can't divide
can be x-linked
earlier tx = better outcome (no tx in 1/2 yrs -> death)
tx = haemopoietic stem cell transplant (gene tx in future?)
NB: increased awareness, early dx with rapid tests, early referral for transplant
can be Dx prenatally (excellent outcome)
medical emergency - immediate management
ensure adequate tx for all infections (consider unusual pathogens, polymicrobial infections)
irradiate all blood products before transfusion (or else likely to be fatal)
no live vaccines
immediate immunological investigations + IG TX
hyper-IgM syndrome (CD40 deficiency)
secondary
HIV
chemo
radio
transplant recipients
Tx for severe autoimmunity
suspect this it patient has shingles affecting multiple dermatomes
assessing T cell function
clinical Hx (ask for HIV risk factors)
lymphocyte counts (incl T cell subtypes, TCR + CK R expression)
delayed hypersensitivity skin test
vaccination with protein antigens
prolif assays
give prophylaxis against pneumocystis jirovecii
in the future
neonatal SCID screening
neonatal stem cell transplants
gene tx
thymic transplants for digeorge syndrome