Immuno EMQ notes Flashcards
Diminished T-cell count, non-funtional B-cells, IL-2 receptor mutation
SCID
Congenital neutropenia
Kostmann’s
Diminished B-cells, IgA/M/G, tyrosine kinase defect, BTK
Bruton’s
Crohn’s leading to loss of proteins and recurrent infx
Protein losing enteropathy
Mutation in CD40 ligand, Inc IgM, def IgA/E/G
Hyper IgM
High neutrophil count, Nitro Blue Tetrazolium (NBT) +ve
Leukocyte adhesion deficiency
Cyclical infx, elastase gene (ELA2)
Cyclical neutropenia
Nose bleeds, easy bruising, GI bleeds, reduced IgM, increased IgA+IgE
Wiskott-Aldrich syndrome
WASp gene mutation
MHC III mutation, low IgG, IgE, IgA
Common variable immunodeficiency Complement
Nitro-blue-tetrazolium test -ve, pneumonia, abscesses, X linked, NADPH oxidase defect
Chronic granulomatous disease
MHC I/II deficiency, sclerosing cholangitis, hepatomegaly, jaundice
Bare lymphocyte syndrome
recurrent chest and diarrhoeal illnesses
Selective IgA def
Granulocyte and lymphocyte deficiency
Reticular dysgenesis
Mouse monoclonal Ab targeted at CD3, used in allograft transplatation
OKT3/muromonab-CD3
Inhibits calcineurin, gum hyperplasia
Cyclosporine A
Anti-metabolite drug used in immunosupressive therapy, purine analogue
Azathioprine
Binds to FKBP-1a
Sirolimus
Binds to FKBP-1a targeting T-cell activation
Tacrolimus
Anti-CCP
RA
Anti-smooth muscle
Autoimmune hepatitis
Anti-jo, rash around eyes, pulmonary effects
Dermatomyositis
Reynaud’s, difficulty swallowing, anti-centromere
Limited systemic scleroderma/CREST
Widespread rash, pulmonary, anti-topoisomerase
Diffuse systemic scleroderma
p-ANCA, medium and small vessel vasculitis
Churg-Strauss
Dry mouth, dry eyes, exocrine destruction, parotid swelling, arthralgia, raised ESR
Sjogren’s
Saddle-shape nose, haemoptysis,haematuria, epistaxis, c-ANCA
Wegener’s