Immuno

Question 1

Bare lymphocyte syndrome (2 types)

Answer 1

T1= absent MHC 1
T2= Absent MHC2
Assoc w/ sclerosing cholangitis, hepatomemegaly + jaundice by 3months

Question 2

DiGeorge

Answer 2

CATCH22 - 22q11

Normal B cells + IgM

Question 3

Kostamnn

Answer 3

Severe congenital neutropoenia

NBT +ve

Question 4

LAD

Answer 4

Neutrophilia + NBT+ve

Delayed cord separation

Question 5

Cyclical neutropoenia

Answer 5

Neutropoenia for 6 days every 3 week
Aut dom (ELA2 mut)

Question 6

CGD

Answer 6

NBT -ve, NADPH oxidase def

X linked

Question 7

Wiskott-Aldrich

Answer 7

Mut in WASp
Low IgM, w/ High IgA+G
Epistaxis + eczema
X linked

Question 8

Reticular dysgenesis

Answer 8

Absolute def in granulocytes + lymphos

Severe neonatal sepsis

Question 9

Chediak Higashi

Answer 9

Protein trafficking defect (microtubules)

Neutropoenia, defective 1o haemostasis+ albinism

Question 10

Complement deficiencies (classical)

Answer 10

CH50 test abnormal

Assoc w/ SLE

Question 11

Complement deficiencies (alt)

Answer 11

AP50 test abnormal

Predisposes to infection w/ encapsulated bacteria

Question 12

Complement deficiencies (common)

Answer 12

Both AP50 and CH50 tests abnormal

assoc w/ MPGN

Question 13

Hereditary angioedema

Answer 13

C1 inhibitor deficiency

Question 14

POEMS

Answer 14

Polyneuropathy, Papilloedema, Pulmonary disease
Organomegaly, Oedema
Endocrinopathy
M protein
Skin abnormalities (hyerpig + hypertrich)

Question 15

HLA DQ2

Answer 15

Coeliac (two eight or not to eat?)

Question 16

HLA B27

Answer 16

Ank spond

Question 17

HLA DR2

Answer 17

Goodpasture’s (DR15)

Question 18

HLA DR3

Answer 18

SLE, Grave’s, MG + T1DM

Question 19

HLA DR4

Answer 19

RA + T1DM

Question 20

PTPN22

Answer 20

RA, SLE + T1DM

Question 21

CTLA4

Answer 21

SLE, T1DM, Grave’s

Question 22

Anti Rh blood group Ag Ab

Answer 22

AIHA

Question 23

Anti RBC Ag Ab

Answer 23

AIHA

Question 24

Anti GlpIIa/IIIb

Answer 24

ITP

Question 25

pANCA

Answer 25

Churg Strauss (eMPA), MPA

Question 26

Anti GBM (T4 collagen)

Answer 26

Goodpasture's (T1 RPGN)

Question 27

Anti gastric parietal cell + Anti IF

Answer 27

Pernicious Anaemia

Question 28

cANCA

Answer 28

Wegener's (T3 RPGN)

Question 29

Anti TSH R Ab (stim)

Answer 29

Grave's

Question 30

Anti AChR Ab

Answer 30

MG

Question 31

Anti dsDNA

Answer 31

SLE

Question 32

Anti histone

Answer 32

Drug induced SLE

Question 33

ANA

Answer 33

SLE, Sjorgren's, AI hep + dermatopolymyosistis

Question 34

Anti Ro

Answer 34

SLE, Sjorgren's, Congenital heart block

Question 35

Anti La

Answer 35

SLE, Sjorgren's (speckled on IF)

Question 36

Anti U1RNP

Answer 36

SLE, Mixed CTD

Question 37

Anti Jo1 (+RNA Synthetase)

Answer 37

Dermatomyositis/Polymyositis

Question 38

Anti CCP

Answer 38

RA

Question 39

Anti GAD

Answer 39

T1DM

Question 40

Anti IA2 + phogrin

Answer 40

T1DM (tyrosine phosphatases)

Question 41

Anti Mitochondrial

Answer 41

Primary Biliary Cirrhosis

Question 42

Anti Centromere

Answer 42

Limited Cutaneous Scleroderma (CREST)

Question 43

Anti topoisomerase/Scl70 + RNA pol 1,2+3 + Fibrillarin

Answer 43

Diffuse Cutaneous Scleroderma

Question 44

Anti Thyroglobulin, Thyroperoxidase

Answer 44

Hashimoto's thyroiditis

Question 45

Anti EMA

Answer 45

Coeliac (cause of dermatitis herpetiformis)

Question 46

Anti robinucleoprotein

Answer 46

Mixed CTD (=Anti URNP1!)

Question 47

Anti Cardiolipin + B2 glp

Answer 47

Antiphospolipid

Question 48

ANA + SMA

Answer 48

T1 AI Hep

Question 49

Anti LKM 1 Ab

Answer 49

T2 AI Hep

Question 50

Anti Soluble liver AB

Answer 50

T3 AI Hep

Question 51

Antiganglioside GM

Answer 51

ALS

Question 52

Antiganglioside LM

Answer 52

Guillain Barre

Question 53

RPGN Types

Answer 53

T1= Anti GBM= Goodpasture's w/ linear stain T2= Immune complex= IgA neph w/ granular stain T3= Pauci-immune= Wegener's/MPA w/ absent stain NB all cause Crescent formation

Question 54

Muromonab (OKT3)

Answer 54

Anti CD3

Question 55

Basilixumab

Answer 55

Anti IL2R

Question 56

Tocilizumab

Answer 56

Anti IL6R

Question 57

Natalizumab

Answer 57

Anti aplha4 integrin

Question 58

Daclizumab

Answer 58

Anti IL2R

Question 59

Alemtuzumab

Answer 59

Anti CD52

Question 60

Abtacept

Answer 60

CTLA4-IG fusion protein blocks costume of T cells

Question 61

Efalizumab

Answer 61

Anti CD11a

Question 62

Ustekinumab

Answer 62

Anti p40 subunit of IL12+23

Question 63

Rituximab

Answer 63

Anti CD20

Question 64

Infliximab

Answer 64

Anti TNF alpha

Question 65

Etanercept

Answer 65

Anti TNF alpha (TNF2R+ IgG1 fusion protein)

Question 66

Denosumab

Answer 66

Anti RANKL in bones

Question 67

Anti Lymphocyte globulin

Answer 67

Tx allograft rejection

Question 68

Adalimumab

Answer 68

Anti TNFalpha - fully humanised