Immuno High Yield Dit

Front 1

X linked recessive defect in tyrosine kinase gene-->

Back 1

Bruton's agammaglobulinemia (pre B cells can't mature; recurrent bacterial infections after 6 months)

Front 2

22q11 deletion assoc'd with?

Back 2

DiGeorge syndrome

Front 3

defect in early stem-cell differentiation, presents with recurrent viral, bacterial, fungal, and protozoal infextions

Back 3

SCID (multiple causes, 1 of which might be adenosine deaminase deficiency)

Front 4

IL-12 receptor deficiency--> ?

Back 4

disseminated mycobacterial infections (no IL-12, no Th1 response)

Front 5

X-linked defect in ability to mount IgM response to capsular polysaccharides of bacteria-->

Back 5

Wiskott-Aldrich syndrome (also inc'd IgA, nl IgE, dec'd IgM; triad: recurrent pyogenic infxns, thrombocytopenic purpura, eczema)

Front 6

what immune definiciency assoc'd with inc'd IgA, nl IgE, dec'd IgM?

Back 6

Wiskott-Aldrich (Xlinked)

Front 7

retained primary teeth, coarse facies, high IgE assoc'd with?

Back 7

Job's syndrome (decd activation of mphages b/c helper T cells no make IFN-gamma; "cold" staph abscesses, eczema)

Front 8

delayed separation of umbilicus + severe pyogenic and fungal infections?

Back 8

Leukocyte adhesion deficiency syndrome (type 1-beta 2 integrin def; type 2--fucosyltransferase gene mutation-required for synth of sialyl-Lewis X, aka CD15s)

Front 9

AR defect in microtubular formation with partial albinism =

Back 9

Chediak Higashi dz (recurrent staph strep infxns, peripheral neuropathy)

Front 10

negative nitroblue tetrazolium dye reduction test

Back 10

Chronic granulomatous dz (lack of NADPH oxidase-->opportunistic bacterial infxns esp S.aureus and e.coli, Aspergillus)

Front 11

IgA deficiency + spider angiomas

Back 11

ataxia-telangiectasia (AR, defects in DNA repair enzymes, cerebellar problems)

Front 12

___ is Ab associated with SLE
___ (2) are Abs specific for SLE

Back 12

ANA
anti-dsDNA
anti-Smith

Front 13

___ is Ab associated with drug-induced SLE

Back 13

anti-histone

Front 14

___ is Ab associated with RA

Back 14

RF (anti-IgG IgM)

Front 15

___ is Ab associated with CREST syndrome
___ is Ab associated with diffuse scleroderma

Back 15

anti-centromere
anti-Scl-70 (anti-DNA topoisomerase I)

Front 16

___ is Ab associated with 1' biliary cirrhosis

Back 16

anti-mitochondrial

Front 17

___ (2) is Ab associated with celiac disease

Back 17

anti-gliadin
anti-endomysial

Front 18

___ is Ab associated with Goodpasture's

Back 18

anti-basement membrane

Front 19

___ is Ab associated with pemphigus vulgaris

Back 19

anti-desmoglein (part of desmosome)

Front 20

___ (2) is Ab associated with Hashimoto's

Back 20

anti-microsomal
anti-TG

Front 21

___ is Ab associated with polymyositis

Back 21

anti-Jo-1

Front 22

___ is Ab associated with dermatomyositis

Back 22

anti-Jo-1

Front 23

2 Abs associated with Sjögren's syndrome

Back 23

anti-SS-A
anti-SS-B

Front 24

___ is Ab associated with mixed CT disease

Back 24

anti-U1 RNP

Front 25

___ is Ab associated with autoimmune hepatitis

Back 25

anti-smooth muscle

Front 26

___ is Ab associated with DM1

Back 26

anti-Glu decarboxylase

Front 27

___ is Ab associated with Wegener's

Back 27

c-ANCA

Front 28

4 B cell-related immunodeficiencies

Back 28

Bruton's agammaglobulinemia
hyper-IgM syndrome
selective Ig deficiency
common variable immunodeficiency (CVID)

Front 29

Bruton's agammaglobulinemia is caused by ___ which prevents ___

Back 29

BTK (Y kinase) defect
B cell maturation

Front 30

___ (2) is normal in Bruton's agammaglobulinemia

Back 30

pro-B cell
pre-B cell

Front 31

hyper-IgM syndrome is caused by ___ on ___
this causes ___

Back 31

defective CD40L
T cells
inability to swich Ig isotypes

Front 32

___ is the most common version of selective Ig deficiency

Back 32

IgA deficiency

Front 33

common risk with IgA deficiency

Back 33

anaphylaxis from transfusion with blood products containing IgA

Front 34

4 T cell immunodeficiencies

Back 34

DiGeorge
IL-12R deficiency
Hyper IgE syndrome
chronic mucocutaneous candidiasis

Front 35

IL-12R deficiency causes deficient ___
___ is a result
___ level is abnormal

Back 35

TH1 response
disseminated mycobacterial infection
IFNg

Front 36

hyper IgE syndrome is aka ___

Back 36

Job's syndrome

Front 37

hyper IgE syndrome is caused by deficient ___

Back 37

IFNg production

Front 38

hyper IgE syndrome pw ___ (5)

Back 38

coarse Facies
cold staph Abscesses
retained 1' Teeth
high IgE
Derm problems (eczema)
(FATED)

Front 39

IL2-R deficiency is linked to chromosome ___

Back 39

X

Front 40

ataxia-telangiectasia pw ___ (3)

Back 40

ataxia
telangiectasia
IgA deficiency

Front 41

Wiskott-Aldrich syndrome is linked to chromosome ___

Back 41

X

Front 42

Wiskott-Aldrich syndrome pw ___ (3)

Back 42

Thromobocytopenia
Infections
Eczema

Front 43

___ (2) is high in Wiskott-Aldrich syndrome;
___ is low

Back 43

IgE
IgA
IgM

Front 44

3 kinds of phagocyte dysfunction

Back 44

leukocyte adhesion deficiency
Chediak-Higashi
CGD

Front 45

hyperacute transplant rejection is caused by ___
it takes ___ to occur

Back 45

preformed Abs against graft
minutes

Front 46

acute transplant rejection is caused by ___
it takes ___ to occur

Back 46

CD8 rxn against graft MHC
weeks

Front 47

chronic transplant rejection is caused by ___ due to ___ (2)
it takes ___ to occur

Back 47

obliterative vascular fibrosis
T cells
Abs
months/years

Front 48

cyclosporine raises risk of ___ (2);
SE is ___;
prevent it with ___

Back 48

viral infection
Ly
nephrotoxicity
mannitol

Front 49

rapamycin is aka ___
mechanism is ___

Back 49

sirolimus
binds mTOR (mammalian target of rapamycin), interfering with IL-2 signalling

Front 50

3 rapamycin SEs

Back 50

hyperlipidemia
thrombocytopenia
leukopenia

Front 51

daclizumab is a ___
it has a high affinity for ___

Back 51

anti-IL-2R mAb
activated T cells

Front 52

IFNa is indicated for ___ (4)

Back 52

HBV
HCV
Kaposi's sarcoma
malignant melanoma

Front 53

IFNg is indicated for ___

Back 53

CGD