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Flashcards in IMMUNO Deck (421):
1

CCL11 other name

CCL11 also known as eotaxin

2

TLR4 coreceptor

CD14

3

what is the rhinovirus receptor on T cells

ICAM1

4

What are the major criteria for the API

parental asthma, sensitization of aeroallergen, Dr dx of AD

5

What are the minor criteria for the API

food sensitization, >4% Eos, wheezing apart from colds

6

What is considered a positive API

one major OR two minor criteria

7

Chemokines important in AD

CTACK (cutaneous T-lymphocyte attracting chemokine; CCL27, TARC (thymus and activation-regulated chemokine)

8

What does TSLP stand for?

TSLP: Thymic Stromal Lymphopoeitin

9

Which cells contain Birbeck Granules

Langerhans cells

10

Prolamin superfamily types of foods and proteins

seed storage proteins, lipid transfer protein, alpha-amylxase/protein inhibitors

11

Cupin superfamily

7s vicillim and 11s globulins

12

PR10 protein family

Bet v 1, birch

13

Atopy leads to increased risk for which agents anayphlayxis?

LIER: latex, idiopathic, exercise, radio contrast (NOT meds)

14

Which agent does pre-treatment not help in anaphylaxis risk?

Latex

15

Which agents does pre-treatment HELP in anaphylaxis risk?

radiocontrast, cold and flueorescien-related anaphylaxis

16

Farmer's lung

thermophilic actinomyteces (sacchropolyspora rectivirgula, thermoactinomyces vulgaris) - moldy hay, grain, compost

17

Bagassosis

Thermoactinomyceses sacchari; moldy sugar cane

18

Humidifier fever/AC lung

Thermoactinomyces (T. Vulgarin, T. sacchiri, T candiddus); Klebsiella, Acanthamoeba

19

Hot tub lung

Mycobacterium avian complex (MAC) or Cladosporium - from contaminated water

20

Basement shower lung

Epicoccum nigrum; mold growing in unventilated showers

21

Malt worker's lung

aspergillus species

22

Cheese worker's lung

Penicillium casei - exposure to moldy cheese

23

Machine operators lung

Pseudomonas, Acinetorbacter, Mycobacter; PAM is a machine operator

24

Bird fancier's lung, pigeon breeder's lung

Avian proteins - exposure to droppings, feathers, serum

25

Detergent worker's lung

Bacillus subtilis

26

Woodworker's lung

Alternaria - moldy wood dust

27

Winegrower's lung

Botrytis cinerea - mold on grapes

28

Summer type HP

Trichosporium cutaneous - contaminated houses, coming in Japan

29

El niño lung

Pezizia - exposure to mushroom fungus after heavy rainfall

30

Saxophonist lung

Candida albicans (Think Bill Clinton has a yeast infection)

31

Wheat weevil lung/miller's lung

Sitophilus - contaminated flour

32

Chemical worker's lung

TDI (toluene diisocyanate), MDI (diphenylmethane diisocyanate)

33

Epoxy Resin worker's lung

Phthalic Acid

34

Plastic Worker's lung

Trimetallitic anhydride

35

Cocamidopropyl betaine

in shampoos, facial cleaners and bath products; one of the MCC contact sensitivity

36

Paraphenylenediame

MCC cause of contact HSN in hair dressers

37

Glycerol thioglycolate

Permanent wave solution; one of the MCC contact sensitivity

38

Ethylenediamine dihydrochloride
What to avoid if sensitive

in topical creams; need to avoid nystatin, aminophylline, and piperazine antihistamines (meclizine and cyclizine)

39

Allergen in Henna

paraphenylenediamine (same as hair dresser contact derm)

40

Only long term/prophylaxis medication for HAE?

Cinryze: plasma derived C1-INH
(I can see the CINRYZE (sunrise) in the long run"

41

Which HAE medication has a risk for anaphylaxis:

Kalbitor (kallikrien inhibitor ecallantide) - "Kalli has anaphylaxis"

42

Live Vaccines Mnemonic

BOYs Love The CRiMe: BCG, OPV, Yellow Fever, Live, Typhoid, Chicken Pox (varicella), Rubella, MMR, Mumps

43

Which two sinuses have the most complications in sinusitis?

sphenoid, frontal

44

Which sinuses are most likely to have polyps?

Ethmoid sinuses

45

Which allergen is CRS w polyps more likely to be sensitive to?

DM

46

Kabuki syndrome

A type of CVID - KMT2D mutation

47

WHIM syndrome mutation

CXCR4 mutation AD

48

Where do neutrophil precursors arrest in severe congenital neutropenia

promyelocyte-myelocyte phase

49

Molecular defect for SCN1

ELANE defect

50

Molecular defect for SCN2

GFII

51

Molecular defect for SCN3

HAX1

52

Molecular defect for SCN4

G6PC3

53

Molecular defect for SCN5

VPS45

54

Which 2 micro bugs is CGD NOT associated with?

Streptococcus PNA, Pneumocystis (PJP) infections

55

Which infectious bugs is CGD associated with? MUST KNOW ALL; also which type of infections? Mnemonic

CATALASE POSITIVE; staph aureus, burkholderia cepacia, serrate marcescens, nocardia sp, aspergillus fumigateurs and aspergillus nidulans; SerMa ASked the Staph at the BerCshires for NOte CARDs for FUMes and Nidus (Serrate marcesens, Asp Fume and Nid, Staph Aureus, Burkholdria Cepacia, Nocardia sp.)

56

CCL5 : aka / receptor

CCL5 aka RANTES -- receptor CCR 1, 3, 5

57

CCL11: aka / receptor

CCL11 aka Eotaxin -- receptor CCR3

58

CCL17: aka / receptor

CCL17 aka TARC-- receptor CCR4
(1+7 = 8 / 2 = 4)

59

CXCL8: aka / receptor

CXCL8 aka IL8 -- receptor CXCR1, 2

60

Spina bifida antigens for latex

Hev b1, Hev b 3

61

Where dies latex allergen come from?

Cytoplasmic exudate of the hevea brasiliensis tree, Hev b 1-13

62

Hev b 2 assoc food?

Bell pepper, olive

63

Hev b 5 assoc food?

kiwi, potato, sugar beet

64

Hev b 6 assoc food?

bananda, avocado, chestnut, sweet pepper

65

Hev b 7 assoc food?

Hev b 7 = "patatin like proteins"; potato and tomato

66

Hev b 13 assoc food?

Potato

67

MCC of biting insect systemic run?

Triatoma - painless bite

68

What do Asain Lady Beetle cross react with?

Asian lady beetle (Harmonia Axydris) - cross reactive with cockroach

69

Order of sinus development? Mnemonic

My Extremely Sweet Friend: Maxillary, Ethmoid, Sphenoid, Frontal

70

Sinus drainage - Mnemonic?

FM AM, PS SS
Frontal, Maxillary, Ant Ethmoid - Middle meatus
Post Ethmoids, Sphenoid - Sphenoethmoid recess=poabove Superior turbinate

71

What does the middle meatus open into?

Hiatus Semilunaris

72

Which mutation is seen in Myeloproliferative HES?

PDGFRB, FGFR1 rearrangements; Deletion of 4q12 --> FIP1L1-PDGFRA fusion

73

What is the mutation and inheritance in familial HES?

5q 31-33

74

Well's syndrome is...

eosinophilic cellulitis

75

How do you treat myeloproliferative HES?

Imatinib: tyrosine kinase inhibitor

76

What is the ligand for CD117? (what is CD117 aka?)

CD117 aka C-kit - ligand is stem cell factor, which is also required for mast cell survival

77

What staining pattern are mast cells identified with?

+ CD2 +/- +CD25, +CD117

78

What is the spirochetal infection causing Lyme disease?

Borrelia burgdorferi

79

What tick does Borrelia burgdorferi infect?

Ixodes ricinus

80

Rx for Lyme?

Doxy, cefuroxime, amoxicillin (>8 years old)

81

Human Monocyte Ehrlichiosis infectious cause?

Ehrlichiosis chaffeensis

82

Principle vector tick for Ehrlichiosis is?

Amblyomma americanum

83

Treatment for Ehrlichiosis?

Tetracycline and chlroamphenicol

84

Human Granulocyte Anaplasmosis infectious agent?

Anaplasma phagocytophilum is infectious agent

85

Human Granulocyte Anaplasmosis vector?

Ixodes scapulars is Human Granulocyte Anaplasmosis vector

86

Infectious agent causing babesiosis?

Babesia microti

87

Babesiosis vector?

Ixodes scapularis

88

Babesiosis symptoms/lab findings

Babesiosis: fatigue, fever; hematologic and liver Abn

89

Is p-anca or c-anca more specific in Wegener's (granulomatosis w polyangiitis)?

C-anca

90

Which vaccines are considered protein vaccines?

Tetanus and Diphtheria (T cell dependent)

91

Which vaccines are considered polysaccharide vaccines?

Meningococcal and Pneumococcal (Pneumovax); T cell independent

92

Which vaccines are considered polysaccharide vaccines?

Meningococcal and Pneumococcal (Pneumovax); T cell independent

93

Which type of Ag do NKT and gamma/delta T cells respond to?

Lipid antigens

94

The meningococcal and pnuemococcal vaccines are considered what type of vaccines?

Polysaccharide; conjugated

95

What are the cytokines of MHC1

IFN alpha, beta, gamma

96

What are the cytokines of MHC2

IFNgamma

97

Which components are part of the MHC1 processing pathway?

proteasome, TAP

98

Calnexin, Calreticulin, Erp57, and tapasin are "chaperone proteins" in which MHC pathway?

MHC1

99

Where on chromosome 6 are TAP proteins encoded?

Class 2 locus

100

Where on chromosome 6 are LMPs from the proteasome encoded?

Class 2 locus

101

What are components of the MHC2 processing pathway?

cathepsins, Ii (invariant chain), CLIP (class 2 invariant chain peptide), HLA-DM

102

What molecule removes CLIP for peptides to bind MHC2?

HLA-DM removed CLIP

103

MHC2 def: what is usually deficient?

transcription factors for MHC2: MHC2TA, RFX5, FRXAP, FRXANK

104

Types of infections in MHC class 1 def?

sinopulmonary, granulomatous skin lesions, necrobiosis lipoidica

105

Types of infections in MHC class 2 def?

diarrhea, hepatosplenomegaly, transaminitis, sclerosis cholangitis (Cyrpto); pulm infxn: PJP, encapsulated bacteria, herpes, RSV; meningitis

106

Treatment of MHC1 vs MHC 2?

MHC2: HSCT; MHC1: aggressive infection Rx

107

What is the inheritance of MHC 1+2?

AR

108

What 2 factors determine lymphocyte tolerance?

Antigen concentration and Antigen affinity determine tolerance; high [ ] and affinity promote negative selection

109

What markers do Tregs express?

Tregs: CD4, CD25, FOXP3

110

What is the other name for CD25?

IL2Ra chain

111

What cytokines do Tregs depend on?

IL2, TGFB; IL10 helps maintain tolerance

112

Which cytokines are released in super antigen stimulation?

IFNg and TNFa

113

What kind of cells express AIRE?

AIRE is expressed by thyme medullary epithelial cells

114

Where are Tregs made?

Thymic medullary

115

What cytokines assist in regulation of Tregs?

IL10, TGFb

116

BAFF

TNF family member, signal for B cell homeostasis

117

What enzyme is class switching mediated by?

Class switching is mediated by AID instead of RAG

118

How much Ig does the adult human produce in 1 day?

2-3 grams of Ig per day

119

Which Ig fixes complement the best?

IgM fixes complement the best

120

Which Ig has the highest total body concentration?

IgA

121

Which Ig has the most daily production?

IgA

122

Best Ig for ADCC?

IgG1

123

IgG subtype with shortest half life?

IgG3

124

IgA1 vs IgA2 in location?

IgA1: serum and respiratory tract
IgAs: lower GI tract

125

Ig with shortest half life?

IgE: 2 days

126

Class switch recombination changes which region: V vc C?

Class switch changes C region

127

Somatic hypermutation changes which region: V or C?

Somatic hypermutation changes the V region

128

Cytokine for T cell stimulation?

IL2 (it's receptor is CD25)

129

Which chromosome is C1-INH on?

Chromosome 11

130

Molecular mimicry: Coxsackie B virus

Glutamic decarboxylase

131

Molecular mimicry: Hep B virus

Major Basic Protein

132

Molecular mimicry: HSV

Ach receptor

133

What are the 5 types of PRR?

LRR, RLR, Ddx, Pyphin, CLR

134

What does TIR stand for?

Toll IL-1R domain (on LRR)

135

Which TLR is triggered by influenza?

TLR10

136

Which TLR is triggered by flagellin?

TLR5 = flaggelin

137

Which TLRs are associated with Heat Shock proteins?

TLR 2, 4, 6 (all extracellular)

138

Which is the TLR that does not use IRAK4?

TLR3 does not use IRAK4/IRAK1/IRAK2

139

What is the next step after MyD88 stimulation in the pathway?

MyD88 --> IRAK4/2/1 --> TRAF6 --> NEMO --> NFkB

140

Which TLR is associated with CD14?

TLR 4 is associated with CD14 (there is a #4 in both)

141

Which NLR is associated with Crohn's disease?

NOD2 is mutated in Crohn's disease

142

What does STING stand for?

STING: Stimulator of INF Genes protein; abn in SAVI

143

What does NALP stand for?

Nacht domain leucine-rich repeat and PYD containing proteins

144

What does RLR stand for?

RNA-sensing RIG-I-like receptors

145

What does SAVI stand for?

SAVI: STING-associated vasculopathy with onset in infancy

146

What kind of mutation leads to SAVI?

GOF mutations of STING leads to SAVI

147

What is the function of Dectin 1/2?

Dectin 1/2 is a CLR receptor: recognize the fungal cell wall B glucan to facilitate mold allergen uptake

148

What is the function of DC sign?

DC sign is a CLR receptor: receptor to facilitate entry for Ara h 1 / Der p 1/2

149

What is the function of mannose receptor?

Mannose receptor is a CLR receptor: recognizes candida; allows for entry of Ara h 1 / Der p 1/2

150

What happens when there is a breakdown CLR anti-candida pattern?

Chronic non-invasive candidate infections

151

What is the difference bw Dectin 1 and CARD9 mutation?

Dectin 1: strictly mucocutaneous
CARD9: some invasive infection, dermatophytes

152

What cells are CD3- and CD56 +

NK cells

153

Which cytokine do NK cells require for DEVELOPMENT?

NK cells require IL-15, EOMES

154

Which HLA is prominent in gestational trophoblasts?

HLA-G (babies say gaga)

155

Which lymphocyte is most prevalent in pregnant decide?

The NK cell

156

Which chromosome is KIR inherited on?

Chromosome 19

157

Which chromosome is MHC inherited on?

Chromosome 6

158

What are the transcription factors needed for ALL ILCs?

NIFL3, TOX, Id2 (NIFty TOXic IDeas)

159

What infections are TLR3 deficiency more prone to?

TLR3 def: HSV encephalitis; influenza cerebrates (CNS inflammation, decrease in IFN-lambda production)

160

What is the inheritance of TLR3 def?

AD

161

What is deficient in production in TLR3 def?

INF alpha, beta, lambda

162

UNC93B def inheritance?

AR

163

UNC93B infections?

HSV encephalitis

164

TRIF/TRAF3/TBK1/IRF3 def infections?

HSV encephalitis

165

What does Dectin1/2 induce in CMCC?

Induces Syk/CARD9

166

Which diseases present with Oculocutaneous albinism?

Oculocutaenous albinism: Chediak Higashi, Griscelli syndrome, Hermansky Pudlak

167

How to differentiate Chediak Higashi, Griscelli syndrome, Hermansky Pudlak ?

BLEEDING in Hermansky Pudlok because of platelet granule defect

168

CNKD1 mutation and inheritance?

GATA2 - AD

169

CNKD2 mutation and inheritance?

MCM4 (mini chromosome maintenance complex 4)- AR

170

FNKD1 mutation and inheritance?

FCGR3A (CD16) - AR

171

What is missing in GATA2 def? CD 56 dim or CD56 bright?

CD56 bright are missing from GATA2 def (CNKCD1)

172

What kind of killing is affected in FNKC def?

ADCC intact; spontaneous cytotoxicity decried; EBV cells not killed

173

What are the Src family kinases?

lyn, lck, fyn

174

What are the Syk family kinases?

Syk, ZAP-70

175

What are the Btk family kinases?

Btk, Itk

176

What are the inhibitory phosphatases of ITIM?

SHP-1, SHP-2, SHIP

177

Generation of which molecule leads to the intracellular increase in Ca in the TCR pathway?

Generation of IP3 (Which is generated after PIP2 is hydrolyzed by PLCg and produces IP3 and DAG)

178

IL-12RB1 mutation leads to which type of infections?

IL-12RB1 def leads to salmonella and atypical mycobacteria

179

What kind of infections does IRAK4 def lead to ?

IRAK4 def: pyogenic infections: Strep pneumo

180

Castleman's disease: hyper production of what and what symptoms?

Castleman's disease: Hyper IL6 productions: fever, microcytic anemia, normal BM, hypo albumin, elevated CRP

181

What type of cell does IL2 promote?

IL2 promotes Treg cells

182

IL5, what does it promote?

IL5 promotes eosinophils and production of IgA Ab

183

Which two cytokines promote IgE class switching?

IL4 and IL13

184

Which cytokines promotes mucus formation?

IL13

185

How does BAFF work?

Upregulates Bcl2 which is an anti-apoptotic protein

186

How does APRIL work?

Upregulates Bcl2 which is an anti-apoptotic protein

187

What is the TSLP receptor?

TSLPR and IL7Ra

188

What is the basophil differentiating cytokine?

IL3 --> basophils

189

What does CD23 (FceRII) bind to (Ig and CD marker)?

CD23 (FceRII) binds to IgE AND CD23

190

What does the basophil activation test evaluate?

Basophil activation: evaluates for CD63 or CD203c

191

What is the tryptase measurement?

>1.2x baseline tryptase + 2 microgram/mL

192

How to test for NK cell cytotoxicity?

Chromium release assay (Cr51); check for CD107a

193

Deficiency of what part of the complement system leads to recurrent encapsulated infections and autoimmunity?

Defects in early complement C1q, C2, C4, C3 --> encapsulated bacteria and autoimmunity

194

Deficiency of what part of the complement system leads to recurrent meningococcal infections?

Defects in terminal complement C5-C9 --> meningococcal infections

195

Which part of the IL2R makes it high affinity?

IL2Ra

196

What IL receptors is gamma chain (gc) a part of?

gc = IL2, IL4, IL7, IL9, IL15, IL21

197

What two components are part of the IL13R?

IL4Ra and IL13R

198

What two components are part of the TSLPR?

IL7Ra and TSLPR

199

IL-18 aka? Which IL is is similar to?

IL18 aka IFN-g inducing factor ; most similar to IL1b

200

Which adhesion marker does IL18 induce?

ICAM1

201

What diseases have elevated IL6?

Castleman's dz; Atrial myxoma; MM; RA, JIA

202

IFNg deficiency results in susceptibility to which types of infections?

M. tb and other intracellular bacteria

203

What receptor does dupilumab act?

IL4Ra which is on IL4 and IL13

204

What are the chemokines for lymphocyte homing?

CCL19/CCL21 --> CCR7

205

What are the chemokines for pro Th2 response?

CCL17/CCL22 --> CCR4

206

What is the function of E selectin?

E selectin: on the endothelium; homing of T lymphocytes to peripheral sites of inflammation; E = extremity (far away/peripheral)

207

What is the function of L selectin?

L selectin; on Lymphocytes and Leukocytes; GLYCAM - to LN HEV; and MadCAM to PMN rolling

208

What is the importance of B integrin family a4b7?

Binds to MADCAM - important for gut homing

209

ICAM1 CD name

ICAM1 = CD54 (think 5=4 = 9, which is almost 10)

210

ICAM2 CD name

ICAM2 = CD102 (has a 2 in the name)

211

ICAM3 CD name

ICAM3 = CD 50 (3 and 5 both prime numbers)

212

ICAM4 CD name

ICAM4 = CD242 (242 has 4 and 4 is a multiple of 2)

213

What does ICAM1 bind?

LFA1, MAC1, rhinovirus

214

What does ICAM2 bind?

LFA1

215

What does ICAM3 bind?

LFA1 and CD18

216

What does ICAM4 bind?

LFA1 and CD18

217

What is the CD name for VCAM1?

VCAM1 = CD106

218

What does VCAM1 bind?

VCAM1 binds VLA4 - vascular

219

What does PECAM bind?

CD31 and CD38 - platelets

220

What is the CD name for PECAM?

PECAM = CD31 (which it also binds, and E looked like backwards 3)

221

What does MadCam bind?

MadCAM binds a4b7 - mucosa

222

What does NCAM bind?

Binds VLA4 (a4b1)

223

What is the CD name for NCAM?

C56

224

What are the important chemokines in allergy?

CCL2, 5, 7, 11; CXCL8

225

What proteins inhibit the formation of MAC?

S protein and CD59

226

Which molecules are considered anaphylotoxins?

C5a, C4a, and C3a

227

What is C3a chemotactic for?

C3a

228

What is the CD name for CR1?

CR1 aka CD35

229

What is the CD name for CR2?

CR2 aka CD21 (#2 in both)

230

What is the other name for CR3?

CR3 aka Mac-1, CD11b/CD18

231

What is the other name for CR4?

CR4 aka gp 150/95, CD11c/CD18

232

What is the other name for CD46?

CD46 aka MCP = membrane cofactor protein

233

What is the other name for CD55?

CD55 = DAF (decay accelerating factor)

234

What is the other name for CD59?

CD = protectin

235

What is the ligand for CR1?

C3b, C4b, iC3b (B1)

236

What is the ligand for CR2?

C3d, iC3b, C3dg (D2)

237

What is the ligand for CR3?

iC3b and ICAM1

238

What is the ligand for CR4?

iC3b

239

What is the ligand for CD46?

C4b and capsid

240

What is the ligand for CD55?

C5b-8 and monomeric C9

241

How is properdin deficiency inherited?

X linked (all other complement def are typically AR)

242

What is the most common complement d/o seen in SLE?

C1q deficiency.

243

What is the most common AR agammaglobulinemia?

mu heavy chain

244

What is a common codominantly inherited blood disorder?

Blood typing (ABO)

245

ADA SCID T/B/NK?

ADA SCID = (T-/B-/NK-)

246

Reticular dysgenesis T/B/NK?

Reticular dysgenesis (T-/B-/NK-)

247

RAG1/2 T/B/NK?

RAG1/2 T-/B-/NK+

248

Artemis T/B/NK?

Artemis T-/B-/NK+

249

Cernunnos and ligase IV: T/B/NK?

Cernunnos and ligase IV: T-/B-/NK+

250

Common gamma chain T/B/NK?

Common gamma chain: T-/B+/NK-

251

IL7Ra: T/B/NK?

IL7Ra: T-/B+/NK+

252

CD3 subunit def: T/B/NK?

CD3 subunit def: IL7Ra: T-/B+/NK+

253

Coronin 1-A def: T/B/NK?

Coronin 1-A def: T-/B+/NK+

254

CD45 tyrosine phosphatase def: T/B/NK?

CD45 tyrosine phosphatase def: T-/B+/NK+

255

What are the 2 SCID with increased radiation sensitivity?

Artemis deficiency and Cernunnos/ligase IV: both of which are T-/B-/NK+

256

What is the abn in reticular dysgenesis?

defect in mitochondrial adenylate kinase2

257

What are the Es of OmEnn syndrome?

Es: Erythroderma, Eosinophilia, Elevated IgE

258

Wiscott Aldrich gene mutation?

WASP gene mutation

259

At what age does alpha fetoprotein increase in Wiskott Aldrich?

Increased alpha fetoprotein after 6-12 months

260

CD40L deficiency inheritance?

Xlinked - CD40L

261

CD40 deficiency inheritance?

Autosomal

262

NEMO deficiency inheritance?

Xlinked

263

Which def one presents with neutropenia: CD40L, CD40, NEMO

CD40L

264

What are the two enzymes in B cells needed for class switching?

AID and UNG

265

How is NEMO inherited?

NEMO = Xlinked

266

XLP1 mutation?

X linked SH2D1A/SAP gene

267

XLP2 mutation?

X linked defect in XIAP gene

268

MAGT def?

XMEN: X linked immunodeficiency with mag defect

269

What is RALD and what mutation is seen?

RALD: RAS Associated Leukoproliferative Disorder: NRAS/KRAS mutation; nil DNTs

270

What is the mutation in APECED?

AIRE mutation

271

IPEX mutation

X linked FOXP3 mutation

272

How is BTK mutation inherited?

BTK = X linked

273

How is mu heavy chain inherited?

AR - the most common of AR agammaglobulinemia

274

What are AD forms of agamm?

E47, AKA, E2A, TCF3

275

What is the function of BTK?

BTK phosphorylates PLCg2 - also present in monocytes and platelets and that is how you can screen for Btk bc these patients have no B cells.

276

CD27+ is on which type of cells

CD27+ are memory B cells

277

CD 27 are part of which cytokines family?

CD27 part of the TNF family of cytokines

278

CD21 aka?

CD21 aka C3d receptor

279

What are the ligands for TACI?

Ligands for TACI receptor on B cells are BAFF and APRIL

280

What is the function of AID?

AID - deaminated cytosine to uracil (C->U); receptor editing function

281

What is the function of UNG?

Removes Us; function in receptor editing

282

How are CD21, CD19, CD81 defects inherited?

AR

283

What class of meds can cause IgA deficiency?

IgA def: anticonvulsants: phenytoin, carbamazepine, valproic acid

284

Which IgG subclass def is assoc with IgA def?

IgG2 subclass def

285

When is the IgG nadir in children?

IgG nadir is about 3-6 months (400mg/dL)

286

What is the inheritance of ELANE mutations?

AD

287

What results from a Lyst mutation?

Lyst mutation: primary and secondary granules fuse - giant granules (which is Chediak Higashi)

288

Which mutations cause lack of phagocyte secondary granules and lack of defensins?

C/EBPe mutation

289

What kind (enzyme positive or enzyme negative) are most common in CDG? ***** ON EXAM

Catalase positive: S3CNAB2 Frany Phil G: Serratia marsecens, Staph aureus, Salmonella, Chromobacter Violaceum, Nocardia spp, Aspergillus spp, Burkholdaria cepacia, BCG, Francisella philomiragia, Granilobacter bethesdens

290

What is the Rx for CGD?

antifungals, abx, INFg, steroids

291

Difference in neutrophil function in LAD1 vs LAD2?

LAD1: defect in adhesion, sticking vs LAD2: defect in rolling

292

What is the cytokine that induces eosinophils?

IL-5

293

What is the cytokine that induces mast cells?

SCF

294

What is the cytokine that induces basophils?

IL-3

295

What are the transcription factors for basophils?

C-EBPa and GATA-2

296

What are the contents of a basophil?

histamine, IL4, IL13, TSLP

297

How are mast cells and basophils different in how they mature?

Mast cells can mature in the periphery; Basophils lease the Bone marrow mature (BB = mature)

298

Can mast cells regranulate?

Yes - mast cells can regranulate

299

Which mast cell type is deeper in the tissue versus more superficial?

Deeper: MCtc; more superficial: MCt

300

Which mast cell responds to C5a?

MCtc (Basophils also respond to C5a)

301

Which mast cell responds to PAF?

MCt

302

Which mast cell responds to opiates?

MCtc

303

Which mast cell responds to substance P?

MCtc

304

Which mast cell responds to antigen?

MCtc

305

MCtc vs MCt: respond to which triggers?

MCtc: responds to more triggers (substance P, Ag, ipiates, C5a); MCt: responds to PAF

306

What cell responds to f-met-leu-phe?

basophils

307

What are the two cytokines tested in basophil activating test?

CD63 and CD203c

308

What is CD63?

CD63 on basophils - protein coated on the inside pf secretory granules

309

What is CD203c?

CD203c on basophils - ectoenzyme, cleaves nucleotides outside the cell

310

What is CD name for IL3R? (to help ID basophils on basophil activating test)

IL3R = CD123

311

What cells do basophils not make?

Prostaglandins

312

Does omalizumab bind free or bound IgE?

omalizumab binds free IgE

313

On which cell foes omalizumab reduce IgE receptors on: mast cells of basophils?

Basophils

314

What type of tryptase is released with anaphylaxis? *****

B tryptase is released with anaphylaxis

315

What are the dx criteria for cutaneous mastocytosis?

1 major + 1 minor; MAJOR: typical skin lesions assoc w Darier's sign. MINOR: bx shows increased mast cells/ activating KIT mutation in bx

316

True or false: Scabies can cause eosinophilia

True, scabies can cause hypereos

317

What is the CD marker for selecting?

CD62 P, E, L

318

In GATA2 def, which CD56 are low, bright or dim?

GATA2 def = low CD56 bright (GATA2 expressed in CD56 bright cells)

319

Common gamma chain inheritance?

X linked

320

What is the side effect of mepolizumab?

Herpes zoster - consider immunizing beforehand

321

Which biologic has adverse side effect of candidiasis?

Secukinumab (used for psoriasis, IL17A is the target) - blocks the IL17 pathway leading to candidiasis

322

Which biologic has a side effect of niesserial infections?

Eculizumab (C5 inhibitor, used for PNH, aHUS), blocks C5 and terminal complement pathway

323

Which biologic has alpha gal picture?

Cetuximab (EGFR blocker), makes IgE Ab to galactose-a-1, 3-galactose, sensitization to the lone star tick

324

Zinc deficiency clinical phenotype?

periorificial and aural (limbs) dermatitis, alopecia (loss of hair) and diarrhea

325

What is acrodermatitis enteropathica?

AR metabolic condition affecting Zinc def: patients have zinc def phenotype: periorificial and aural (limbs) dermatitis, alopecia (loss of hair) and diarrhea

326

What is the MAO of Rapamycin?

mTOR/AKT blockage

327

Which medications are calcineurin inhibitors?

Tacrolimus, Sirolimus, and Pimecrolimus

328

Mnemonic for LIVE vaccines

LMNOP RSV; ROME Is MY Best Place To go

329

Felty syndrome

RA, Neutropenia, and Splenomegaly (FELTY RANS)

330

How does location of eczema differ from infants/small children to adults?

Infants/small children: face, scalp, extensor (side opposite the joint) surfaces ; Adults: flexural surface

331

What is the genetic defect in Comel-Netherton Syndrome?

SPINK5 - which encodes LEKT1

332

LL-37

cathelicidin, antimicrobial peptide that is decreased in eczema; important in antiviral (think eczema herpeticum and vaccinatum)

333

Which coxsackie strain is related to eczema coxsackium

coxsackievirus A6

334

What is eczema vaccinatum?

disseminated live vaccinia virus from smallpox vaccination (no one gets smallpox anymore in US because it's been eradicated)

335

Which antimicrobial peptides are NOT unregulated in AD but should be?

LL-37 (cathelicidin) and HBD2-3 (human B-defensins)

336

Which two cold urticarias have a low CH50?

low CH50: cyroglobulinemia and leukocytoclastic vasculitis

337

Systemic atypical acquired cold urticaria

Localized or generalized urticaria with cold exposure; negative

338

Cold dependent dermatographism

Dermographic whaling after stroking pre-cooled skin

339

Cold-induced cholinergic urticaria

Exercise in cold environments leads to generalized urticaria

340

Delayed cold urticaria

Urticaria develops 12-48 hours after application of cold provocation

341

Localized cold reflex urticaria

Positive immediate urticarial response at a site distant to cold provocation

342

FCAS: Fam cold auto-inflamm sx

Pseudourticaria, fever, arthralgia, conjunctivitis occurring 1-2 hours after cold exposure; AD mutation in NLRP3

343

PLAID: Phospholipase Cg2 associated def and immune dysregulation

Cold urticaria in childhood, Ab def, autoimmunity. Positive Evaporative cooling test

344

How to physically test for PLAID?

Positive evaporative cooling test; neg. ice cube test

345

What is the mutation in PLAID?

mutation in PLCG2

346

Which type of solar urticaria is assoc with erythropoietic protoporphyria ?

Type 6 VI

347

Which type of urticarias can he passively transferred by serum?

Acquired Cold (IgE, IgM, IgG, cryoglobulins), Solar (Type 1 and 4) and Dermatographism (IgE) (ADS)

348

Factor I def.

Ar, urticaria, decrease Factor I (C3b inactivator), dec C3 levels

349

What is SERPING1?

SERPING1 = C1-INH gene in hereditary angioedema

350

Treatment in Giant cell arteritis?

STEROIDS!! Need to decrease risk for visual loss

351

What is the treatment for Kawasaki disease?

IVIG x 1 and aspiring: decrease the risk of aneurysms

352

What diseases can polyarteritis nodes be associated with?

Hep B or Hep C

353

Which HLA is associating with Abacavir Sn?

Abacavir Sn in white = HLA-B*5701

354

Which HLA is associating with Carbemazepine Sn in Han Chinese?

Carbepazepine in Han Chinese = HLA-B*1502

355

Which HLA is associating with Carbemazepine Sn in Europeans?

Carbemazepine Sn in Europeans = HLA-B*3101

356

Which HLA is associating with allopurinol reactions in Asians?

allopurinol reactions in Asians = HLA-B*5801

357

Which HLA is associating with insulin, gold, and penicillamine reactions?

HLADR3

358

Which cytokine is produced by the T-cells in AGEP?

IL8 / CXCL8

359

Major determinant for PCN allergy

Major: Benzylpenicilloyl

360

Minor determinant for PCN allergy

Minor: Pcisilloate, penilloate, benzylpenicillin (Pen C)

361

Which cytokine is related to mucus gland hypertrophy?

IL9 --> mucous gland hypertrophy

362

Which sinuses drain into the middle meatus?

Anterior ethmoid, frontal, maxillary

363

Where is the OME complex located?

OME complex is located under the middle meatus, and that is why the Anterior ethmoid, frontal, maxillary all drain into the OME/middle meatus area

364

Where do the posterior ethmoids drain?

Superior meatus

365

Which sinuses do polyps usually arise from?

polyps usually arise from the ETHMOID sinuses

366

Do anti-leukotriene agents block the early or late phase?

anti-leukotriene block the early and the late phase

367

Do antihistamines block the early or late phase reaction?

Early and Late phase reaction

368

What CT finding is seen in empty nose syndrome?

turbinates are very small

369

Where is a haller cell located?

Haller cell = medial floor of the orbit (basically next to the nose and under the eye.

370

What is the cell type in Western nasal polyps?

Eosinophilic

371

What is the cell type in Asain nasal polyps?

Neutrophilic (elevated IL8)

372

What is the first line treatment for acute rhinosinusitus?

Augmenting; then doxy or a resp flouroquinolone (moxi)

373

How long does it take after pet removal for allergen to go back to normal levels?

about 4 months (16-20 weeks)

374

Which nasal spray is ok to use in pregnancy?

Budesonide; B=baby (cromolyn is also safe in pregnancy)

375

Which immunosuppressed group can receive the varicella vaccine?

Isolated B cell deficiency

376

What is the rule for antibody containing blood products and vaccines?

If a patient has recently received Antibody containing blood product, they must wait 3 months before receiving MMR and varicella (except zoster) containing vaccines. If patient got vaccine first, wait 2 weeks to give Ab product.

377

What is the CI for getting Rotavirus vaccine?

CI are: immunodeficiency and h/o intussusception

378

What is the vaccine rule for nonfunctional spleen?

Functional or anatomic asplenia: patient should have PCV13 and Menactra 4 weeks apart (cannot get them on the same day like other patients)

379

What is the timing rule for live vaccines?

Either get the live vaccines on the same day, OR get them 4 weeks apart.

380

What are exceptions to the live vaccine rule?

Rule is: get same day or 4 weeks apart; but can get Yellow Fever <30 from MMRV containing vaccines

381

What if a line vaccine is given < 4 weeks/24 days from last live vaccine?

Will need to repeat dose of 2nd vaccine given bc the response probably was not robust enough

382

Which vaccines contain yeast?

Hep B (any Hep B containing vaccine)

383

Which vaccine is contraindicated in egg allergy?

Yellow fever (MMR and influenza are not)

384

Which vaccines contain Gelatin?

HIZ MYV: HepB, Influenza, Zoster, MMR, Yellow Fever, Varicella

385

What mutation confers HIV resistance?

CCR5 deletion of bp32 confers resistance to HIV: dbl mutation = resistance, single mutation = slower progression

386

What is lipocalin also known as?

lipocalin aka calycom

387

What is a direct bronchoprovocation test?

Methacholine challenge

388

What is an indirect bronchoprovocation test?

Exercise, cold air, mannitol, adenosine monophosphate

389

What is considered a positive methacholine challenge test?

< 4 +; 4-16 is borderline

390

Which cells make IL-12?

IL12 is made by myeloid cells, but not T cells or innate cells

391

Conventional dendritic cells CD markers

CD1, CD11b/c, CD13, CD14

392

Plasmacytoid dendritic cells CD markers

CD123

393

Langerhans cells CD markers

CD11c, CD207

394

Interstitial dendritic cells CD markers

CD11c, CD2, CD9, CD68

395

Which lipid mediator is lower in AERD after COX inhibition, leading to bronchospasm?

PGE2 production goes down after COX1 inhibition, and there is no block of 5LO, leading to uncontrolled production of cysLT

396

What is higher in the urine of AERD patients?

LTE4 (because of uncontrolled production of cysLT

397

Which CD markers doe basophils have?

CD123, CD203c, CD63, CD69

398

Which cells produce PGD2?

Only mast cells produce PDG2

399

Platelet Activating Factor: preformed, lipid, or cytokine?

lipid

400

heparin: preformed, lipid, or cytokine?

preformed

401

chondroitin sulfate E: preformed, lipid, or cytokine?

preformed

402

chymotryptase: preformed, lipid, or cytokine?

preformed

403

carboxypeptidase: preformed, lipid, or cytokine?

preformed

404

What is the gene mutation in Familial Cold AutoInflammatory Syndrome?

CIAS1: Cold Induced Auto-Inflammatory Syndrome 1 gene

405

What is the gene mutation in Muckle Wells Syndrome?

CIAS1: Cold Induced Auto-Inflammatory Syndrome 1 gene

406

Which cat allergen is related to Pork-Cat syndrome

Fel d 2: albumin

407

Where does superantigen bind on the TCR?

Vb chain

408

Do superantigen bind to MHCI or MHCII?

Superantigen bind to MHCII (extracellular bacteria)

409

What are the Staph superantigens?

SEA, SEB, (staph enterotoxins); TSST (toxic shock syndrome toxin)

410

What are the strep super antigens?

SPE-A (Strep Pyogenes exotoxins)

411

Where are cells that express AIRE?

Medulla: Thymic Medullary cells

412

Where do Tregs develop?

Tregas develop in the thyme medulla

413

Which TLR binds to mycoplasma?

TLR6/2 - ligand is diacyl lipopeptides

414

Which TLR binds to synthetic sources?

TLR 7/8 - imidazoquinoloones, synthetic, virus

415

Which TLR binds to unmethylated CpG motifs of bacteria and viruses?

TLR 9

416

Serum Sickness is which type of HSN d/o?

Serum sickness if type 3

417

Which medications do you have to be careful of fluticasone causing Cushing's?

Protease Inhibitors - namely Ritonavir

418

Which PID is associated with fractures?

Hyper-IgE/Jobs/STAT3 deficiency

419

What is the major mouse allergen?

Lipocalin: Urine of the male mouse

420

In which eye disease can you see keratoconus?

AKC

421

In which eye disease can you see anterior cataracts?

AKC