Immunology Flashcards
Hallmarks of immune deficiency
SPUR infections
Serious Persistent Unusual Recurrent
Phagocyte deficiences cause
recurrent infections, in unusual sites, can be unusual bac (Burkholderia -this and CF only) or fungi infections
Reticular dysgenesis
no neutro, baso or eosinos or platelets
fatal unless bone marrow transplant
sufferers have a small thymus
Cyclic neutropaenia
neutropenia every 4-6wks
Kostmann syndrome
rare autosomal recessive severe chronic neutropaenia
presents w recurrent infections within 2 wks of birth
Leukocyte adhesion deficiency characterisitics
rare - CD18 leukocyte integrin deficiency recurrent bac and fungal infections v high neutro count deep tissue infections NO PUS mouth infections
Failure in this causes mild disorders
pathogen recognition - as receptors functions overlap
Failure of oxidative killing mechanisms =
chronic granulomatous disease
no O2 respiratory burst can’t clear org.s -> chronic inflam
Test to diagnose chronic granulomatous disease
NBT (nitroblue tetrazolium) - give E. coli and dye sensitive to H2O2 and there will be no colour change
Commonest for of chronic granulomatous disease
X linked p47phox component of NADPH oxidase deficiency
defects in immune components activating others =>
tb reactivation, atypical mycobacterium infection, salmonella
SCID (defects in lymphoid precursors) characteristics:
unwell by 3 months, graft v host skin disease,
FtT, diarrhoea
commonest form of SCID
X-linked - mutation of component of IL-2 receptor
Can’t respond to cytokines NK and T dont develop and B cells immature
low/no T, normal/raised B
Defect in Thymus development
DiGeorge 22q11
DiGeorge characteristics:
FLK, cleft palate, cardiac problems, hypocalcaemia (seizures)
recurrent viral fungal and bac infections
T lymphopaenia, normal of increased B but decreased Igs
Antibody deficiencies cause ___ infections
recurrent bac infections and AI diseases
T cell deficiencies characteristics
BCG infection after vaccination tb and atypical pneumonia deep fungal infections recurrent infections Malignancy in youth AI
Bruton’s X-linked hypogammaimmunoglobulinaemia
no B cells in blood, no plasma cells, no circulating Ig after 6 months
Selective IgA deficiency
recurrent RTIs
Can cause false negative of coeliacs test
CVID
low IgE/A/G, AI often, granulomatous disease, recurrent bac infections, often persistent sinusitis, GI infections, bronchiectasis
Type 1 hypersensitivity reactions =
Allergic/Immediate/IgE mediated response to external antigen
Type 2 hypersentitivity reactions =
Direct cell killing
Type 3 hypersensitivity reactions =
Immune complex mediated
Type 4 hypersensitivity reactions =
Delayed/ Tcell mediated