A/i Boards 2017!!!

Front 1

Severe neutropenia shows up with which infections?

Back 1

skin infections, stomatitis

otitis media

Front 2

Differential for neutropenia?

Back 2

1. Virus or Medications

2. Splenomegaly!!

3. Schwachman-Diamond

4. Cartilage-Hair Hypoplasia

5. Lysosomal disease (Chediak-Higashi LYST, Griselli RAB27a, & Hemansky-Pudlak ADTB3a)

6. WHIM CXCR4

Cyclic neutropenia (ELA4 mutation, mono reverse),

Chronic Benign Neutropen (200-1500), mild inf

Neonatal Isoimmune Neut (NIN), like RH

Kostmann HAX (Familial Severe Neut), AR

AutoImmune Neutropenia of Infancy

Front 3

Autoimmune LymphoProliferative Syndrome (ALPS):

- clinical symptom

- underlying issue

- labs would show

Back 3

-sx: cytopenia with fevers

-Issue: failure of Fas-L (CD178) to induce lymphocyte apoptosis. Lymphs multiple and can suppress other cell lines (low RBC, neutrophils, plt)

- Labs: increased double negative alpha/beta T cells (CD3+, CD4-, CD8-), increased Vit B12

Hint 3

You reach double negative degrees in the ALPs and need vitamin B, Fast!! Otherwise you get cytopenias with fever!

Front 4

Name some chromosomal breakage syndromes.

Back 4

Chromosomal breakage (FYI: photosensitive):

1. Ataxia telangiectasia (cytopenia with fever)

2. Nijmegen (similar to A-T)

3. Bloom's syndrome (short, photosens, unusual face)

4. Fanconi anemia

5. * hypothyroidism

Front 5

What is Chediak Higashi syndrome?

How is Griselli different?

Back 5

AR mutation in CHS (aka LYST) gene

Affect lysosomal trafficking regulator protein, which leads to a decrease in phagocytosis.

clinical picture: CH ANNN

- albinism

- NK cell phagocytosis prob

- neutropenia possible

- neuropathy

In CH, you see giant azurophilic granules that lack proteins. In Griselli RAB27a, you don't.

Front 6

HAE treatment(s) that block kallikrein?

Brands?

Back 6

C1 inhibitor (chronic Cinryze, acute Berinert, recombinant Ruconest)

ecallantide (Kalbitor)

Front 7

HAE treatment that blocks bradykinin B2 receptor?

Brand?

Back 7

Icatibant (Firazyr)

Front 8

What chemokine is in follicular zones? Receptor?

Back 8

CxCR5 bind CxCL13 in follicular zone

Front 9

Most potent chemoattractsnt for neutrophils and it receptor?

Back 9

Neutrophil CXCR2 binds IL-8 (CXCL-8)

Hint 9

Neutrophil X2 is IL8erate (illiterate)

Front 10

WHIM syndrome stand for?

Mutation? Ligand?

Inheritance?

Back 10

Warts, Hypogam, Inf, Myelokathexis.

gof CXCR4 mutation - binds CXCL12

Autosomal Dominant

Also get neutropenia

Neutr CxCR2 : CXCL8 (IL-8)

WHIM CxCR4 : CxCL12, (FYI, CXCR4 is also on HIV)

CxCR5 : CxCL13 Follicular

Front 11

This VIT is standardized for?

Action?

Med that can block?

Back 11

Phospholipase A2 (Api m 1 in honey bee)

-Note: note x-reactive with vespid PLA1

PLA2 produces arachidonic acid from phospholipids. Blocked by glucocorticoids.

Front 12

In this disease, there is a biphasic increase and decrease of CD1a+ Langerhans DCs in the epidermis.

Back 12

Norwegian Scabies

Front 13

What is metachromasia, and which immune cells are defined by it?

Back 13

Color change different from stain.

Mast cells and basophils

Front 14

What is the only X–linked complement component deficiency?

Back 14

Properdin deficiency

Front 15

What is the name for mountain cedar allergen?

Back 15

Jun a ___ (Juniperus ashei)

Front 16

Most common allergen induced immediate hypersensitivity in the Mediterrean?

Back 16

Par j 1
(Parietaria judaica,
sticky weed)

Front 17

Is there cross–reactivity between cockroaches?

Which cockroach allergen is related to animal danders?

Back 17

Yes, but only between Bla g 1 and Per a 1

Bla g 4 (lipocalin–like protein)

Front 18

Allergen common btw cockroach, mites and shrimp?

Back 18

Tropomyosin:

Per a 7 (cockroach)

Pen a 1 (shrimp)

Der f/p 10 (DM)

Hint 18

perpender

Front 19

What is the most common cause of HP in Mexico?

Back 19

Pigeon breeders disease

Front 20

EGPA (Churg–Strauss):

1. stands for?

2. six ACR criteria for diagnosis?

3. definite diagnosis?

Back 20

1. Eosinophilic Granulomatosis with PolyAngitis

2. Eos>1500, Extravas eos

Neuropathy,

Paranasal sinus abnormalities, Pulmonary (migratory) infiltrates,

Asthma

3. DX: eosinophilic vasculitis on lung biopsy

Hint 20

Think ENPA EE/N/PP/A

Front 21

Neutrophils

1. Growth factors

2. cytokine that stimulate PMN?

3. cell products & unique protein

Back 21

GM–CSF, G–CSF

IL-8 (CXCL8)

LTB4

cathepsin G (unique to neutrophils)

Front 22

Monocytes/Macrophage

1. Growth factor?

2. maturation site?

3. cell products?

Back 22

1. GM–CSF

2. Mature in BM, but diff into macrophages and DC in tissues

3. LTB4, LTC4

Front 23

Eosinophils:

1. Growth factors?

2. maturation site?

Back 23

1. IL–5, IL–3, GM–CSF

2. bone marrow

Front 24

Cell products of eosinophils in:

1. Primary

2. Secondary granules

3. Lipid bodies

(Which can selectively release products)?

Back 24

1. Charcot leyden cystal (lysophospholipase)

2. Cationic proteins (MBP, ECP, EDN, EPO),

Enzymes (lysozyme, elastase, cathepsin),

Cytokines

3. COX, LPO, LT

Secondary can selectively release granules.

Front 25

Basophils:

1. Growth factor? & receptor?

2. Maturation site

3. cell products?

Back 25

1. IL–3 (rec is CD123 denim, also found on plasmacytoid DC)

IL–4, IL–5, GM–CSF, nerve growth factor (NGF)

2. bone marrow

3. histamine, chondroitin A, LTC4

Hint 25

Ill Mark received by CD denim on basophils and plasmacytoid DCs

Front 26

Mast cells:

1. Growth factor

2. Maturation site & granule types MCt vs MCtc

Back 26

1. Stem Cell Factor (aka c–kit ligand)

2. MCt: gut, lung (scroll-like granules)

MCtc: gut, skin/eyes, vessels, synovium (lattice/whorled granules). Also has C5a Receptor (CD88).

Recall, C5a is a powerful chemotactic factor for neutrophils.

Hint 26

HCL TPH + CCC

Front 27

Mast cell

1. Pre-formed mediators?

2. Other Cell products MCt vs MCtc

Back 27

1. Pre-formed:

- histamine,

- proteases (tryptase, c/c/c)

- proteoglycans (hep, chon)

- TNF-alpha

2. both MCt/MCtc: (histamine, chondroitin A/E, LTC4),

tryptase, PGD2, heparin, IL-5

MCtc: chymase, carboxypeptidase, cathepsin G

Front 28

agent responsible for Enzyme/Detergent Worker’s Lung

Back 28

Bacillus subtilis enzymes (HMW, IgE-mediated)

Front 29

This is the most common parasite causing Loffler’s syndrome

Back 29

Ascaris lumbricoides

Front 30

Solenopsis invicta and Solenopsis richteri do NOT share which antigen?

Their venom contain 95% what?

Back 30

Sol i 4 is unique.

piperidine alkaloids

Front 31

genus?

names of related genuses?

Most important venom antigen?

US allergen standardized to?

Back 31

DolichoVespula ( hornet, horns blow air)

Vespula (jackets) lay on the ground (ground nests)

Polistes (paper wasp)

Antigen 5 (ves v 5) is the major allergen, but extracts are standardized to hyaluronidase (ves v 2, api m 2, pol a 2)

Front 32

Ataxia–Telangiectasia:

1. first clinical manifestation?

2. characteristic labs & Ig?

3. underlying problem

Back 32

1. Ataxia (walking then prog clumsy)

2. Elevated AFP, CEA, IgM (low IgA & IgE)

3. ATM is PI3 kinase, responsible for DNA repair

Front 33

7 month old girl got pneumonoccal Meningitis despite her PCV-13 vaccine.

Most likely has deficiency in:

TRIF, MyD88, NEMO, or UNC93b???

Back 33

Answer is MyD88

NEMO is Xlinked.

TRIF & UNC93b has HSV encephalitis.

Front 34

DDX for chronic mucocutaneous candidiasis?

Back 34

DOCK8 HIES

AIRE

MHC II defects

STAT1 gain of function

STAT3 / TH17 / IL-17 / Dectin/CARD9

Hint 34

yeasty DAMSSdc

Front 35

MSMD (Mendelian Susc to Mycobactium Disease)

1. kind of infections

2. mutations involved

Back 35

1. MLVSH mycobacterium (disseminated), salmonella, listeria

histoplasma/cocci,

viruses (herpes family, RSV)

MC: IL-12Rb-1 deficiency,

osteomyelitis: IFN deficiency

E. Asian women: IFN auto ab

STAT1 gof/defic,

Tyk 2 HIES,

GATA2 MonoMac, low NK bright

XL-NEMO

Recall, IL-12/23 blocker is ustekinumab.

Hint 35

mycobacterium STIIIG X

Front 36

Abscesses and post-pneumonia formation of pneumatoceles leading to Aspergillus, Primary teeth retention, Classic facies > 16 yo. Other: eczema, mucocutaneous candidiasis (not STAT1 gof or AIRE). Dx?

Back 36

Job/STAT3 deficiency

Post-PNA pneumatocele also in myeloperoxidase deficiency

Front 37

How is hypereosinophilia defined?

HES?

Back 37

Peripheral eo >1500/mcl on 2 occasions,

OR tissue eo + marked peripheral eos.

(% is NOT useful)

HES is when there is end-organ damage 2/2 eosinophilia.

Front 38

Hypereosinophilia + interstitial nephritis: which meds most likely culprit?

Back 38

cephalosporins and PCNs

recall, cefaclor serum sick kids

recall, PIE lung = NSAIDs, nitro

Front 39

Infectious causes of eosinophilia?

Back 39

1. Parasites

2. Fungus

3. Insect (scabies sensitization)

4. protozoa: only Isospora belli & Sarcocystis

Front 40

What is Gleich syndrome?

Back 40

Episodic Angioedema with episodic Eosinophilia (EAE)

Front 41

DDX (2) for T-B-NK- SCID?

Back 41

ADA deficiency (bone anomalies)

& RD (Reticular Dysgenesis)

PNP is also T-B- but can be NK+/-

Front 42

DDX (6) for T-B- (NK ok) SCID?

Back 42

1. RAG1&2 (NO radiation sens!)

2. recombinase repair issues:

- Artemis, Cernunnos, Ligase 4 (radiation sensitive)

3. Nijmegen breakage

4. Omenn!!!

5. PNP, which can also be NK+/-

Hint 42

Think VDJ defects.

Front 43

DDX (2) for T-NK- (B ok) SCID?

Back 43

XL: Common gamma chain (CD 132 demon)

AR: JAK3 deficiency

Recall: CD131 timid is common beta chain

Front 44

Underlying problem in Chronic Granulomatous Disease (CGD)?

Inheritance?

Describe DHR lab

sx & Infections? Why?

Back 44

Neutrophils can't do NADPH-oxidative burst.

65% XL, but rest are AR

DHR: peak don't move (XL dz), 2 sharp peaks (XL carrier), small hill (AR)

Angry bloody diarrhea like IL-10 & NEMO.

Catalase (+) breaks down bacteria H202 so CGD can't borrow.

N BBASSS cfg

- Nocardia

- Burkholderia cepacia

- BCG,

- Aspergillus

- Staph aureus

- Serratia marcenans

- Salmonella

Other:

- Chromobacterium

- Francisella

- Granulibacter

Front 45

Describe MHC Class 1 vs 2 presentation & cross-presentation.

Back 45

MHC 1 (HLA ABC): intracell protein --> TAP to MHC 1 inside ER and export by Golgi/phagosome.

MHC 2 (HLA DQ,DR,DS): extracell phagocytosis --> merge with MHC2 and export by Golgi/phagosome

Cross: endosome fuse with MHC1 on Golgi/phagosome; important for extracellular viruses and tumors

Hint 45

TAP "in" 1ABC (HLA ABC)

"ex" 2Dqrs (HLA Dq,Dr,Ds)

Cross: "ex" 1

Front 46

DDX for HIES (hyperIgE syndrome)

Back 46

AD: Job/Stat3 deficiency

AR: DOCK8, Tyk2, Wiscott-Aldrich, Omenn

Front 47

Name 5 syndromic immunodeficiencies

Back 47

Job STAT3 deficiency

Wiscott-Aldrich

Ataxia Telangiectasia

DiGeorge (+/- immunodefic)

STAT5b (IPEX-like with Treg issues)

Front 48

CD defects in LAD?

Sx and blood group?

Why infections?

Back 48

LAD1: CD18 (pairs with CD11 to form integrins). Think chronic necrotic lesions mos/yrs (recall D-ADA2 also has necrotic lesions).

LAD2: FUCT cannot fucosylate CD15 (sialyl lewis x)...has Bombay blood group & developmental abnormalities.

Neutrophils problem! PMN can't extravasculize from blood vessels.

Front 49

Immunodeficiency with dwarfism?

Back 49

STAT5b (IPEX-like with Treg issues)

Front 50

Causes of agammaglobulinemia?

Back 50

1. XL BTK (phos PLCγ2)

2. μ (IgM) Heavy chain

3. CD79 (Igα, Igβ)

4. CD159 (Surrogate light chain: V pre-B, lambda 5),

5. BLNK (SLP–65) deficiency

6. Good thymoma w/ eosinopenia

7. Other: LRRC8, PIK3r1, TCF3, monosomy 7, trisomy 8)

Front 51

Infections with antibody/humoral deficiency

Back 51

1. sinopulmonary inf by encapsulated bacteria

2. atypical: mycoplasma, ureaplasma

3. GI tract bacteria/virus/protozoa (Giardia)

Front 52

How to dx CVID?

Back 52

1. <2 SD for IgG and (IgA or IgM)

2. Absent isohemaglutinins

3. "poor" vaccine response to protein & polysaccharide

4. exclude other causes

Front 53

DDX for CVID hypogam?

Back 53

ICOS, TACI, BAFF (TNFRSF13c), CD19/20/21, CD81(TAPA-1),

Other: TWEAK, PIK3CD (PI3 kinase), PIK3R1, LRBA, NFkB2, PRKCD, KMT2D (Kabuki), Good thymoma w/ eosinopenia

Front 54

other DDx for hypogam besides CVID (i.e. selective Ig deficiency)?

Back 54

WHIM

PLAID

Medications, infection, protein loss, BM failure / cancer

Front 55

Cutoff for selective IgA deficiency?

Which meds may lead to permanent deficiency as well?

Back 55

<7 mg/dl (in 4 year old or older)

Anti-epileptics

Front 56

For lab lymph proliferation, how many days for mitogen vs. antigen?

Back 56

mitogen: 3 days

antigen: 6 days

Front 57

Clinical lab significance of CD107a?

Back 57

look for this intracellular marker to be expressed extracellularly as a marker of CYTOTOXICITY!

Front 58

BTK is expressed inside which cells?

Back 58

B cells, monocytes, platelets

Front 59

Define lymphopenia generally?

About how much should be T cells, B cells, and NK cells?

Back 59

ALC < 1000/m3 (adults)

ALC < 3000/m3 (infants)

T cells is 65-76% of ALC

NK: 10-15%

B: 10-15%

Front 60

Prevnar vs Pneumovax

Back 60

Prevnar 13 is conjugated with peptide.

Pneumovax 23 has just the carbohydrate capsule.

Front 61

Which tryptase is released from mast cells constitutively?

VS during degranulation?

Back 61

constitutive: Pro-alpha and pro-beta monomers tryptase

degranulation: Mature tryptase (beta tetramers)

Total tryptase = monomers + beta tetramer

Front 62

-mab nomenclature:

target? + source?

Back 62

target:

-li- or -lim- (immune)

-tu- or -tum- (tumor)

source:

-amab (rat)

-emab (hemster)

-imab (primate)

-omab (mouse)

-umab (human)

-zumab (humanized)

-ximab (chimera)

Front 63

IVIG:

- target trough?

- when to check trough?

- side effects on?

Back 63

>500-750 mg/dl to keep serious infections <2.5 yr; (Higher if bronchiectasis!)

after 2-3 infusions to get "accurate" level

on: headache,

off: fatigue before next infusion

Front 64

(AD) PLAID: Stands for?

1. Underlying prob?

2. Clinical sx?

3. Tx?

Back 64

PLc-gamma-2 Antibody defic + Immune Dysregulation

1. PLC-gamma 2 mutation, causing diminished signalling at cold temp. (recall, PIP2->DAG/IP3

2. cold urticaria with evaporative cooling, antibody deficiency, autoimmunity

3. high dose antihistamines +/- IVIG replacement

Front 65

(AD) CAPS: Stands for?

1. Underlying prob?

2. Clinical sx with each type?

3. Tx?

Back 65

CIAS1-Associated Periodic Syndrome

1. CIAS1/NLRP3 (AD) activating mutation of cryopyrin

2. All have Fever + urticaria-like (Neutrophilic, not Eos)

- FCAS (AD): sx with generalized cold exposure

- MW: limb pain, hearing, systemic amyloidosis

- NOMID/CINCA: arthritis, meningitis, epiphyseal overgrowth

3. IL-1 blocker

Front 66

(AD) PAPA: Stands for?

1. Underlying prob?

2. Clinical sx?

3. Tx?

Back 66

Pyogenic Arthritis + Pyoderma Gangrenosa + Acne

1. PSTPIP1 (AD), aka CD2 bind protein 1, which (+) pyrin

2. (see above) terrible

3. no good tx. ???TNF or IL-1 blockers, steroids

Front 67

FMF: Stands for?

1. Mutation? Underlying prob?

2. Clinical sx?

3. Tx?

Back 67

Familial Mediterranean Fever

- MEFV (AR), codes for pyrin

- 1-3 days fever, peritonitis (sterile), +/- pleurisy, arthritis/algia, erypsipelas, rare amyloidosis

- tx: colchicine (blocks pyrin activation), IL-1 rec inhibitor anakinra

Front 68

PFAPA: Stands for?

1. Underlying prob?

2. Clinical sx?

3. Tx?

Back 68

Periodic Fever with Aphthous ulcers, Pharyngitis, and Adenitis

- ?genetic basis

- last 3-6 days, very clockwork

- tx options: outgrow, T&A, steroids (though will make sx more frequent), anakinra

Front 69

HIDS: Stands for?

1. Underlying prob?

2. Clinical sx? Trigger?

3. Population

4. Tx?

Back 69

HyperIgD with Periodic Fever Syndrome

2. MVK (AR) mutation, decrease geranylP somehow lead to increased IL-1 and IgD (not sig)

2. 3-7 days F, abd pain, arthritis/algia, cervical LAD, aphthous ulcers. Vaccines can trigger.

3. Northern Europeans

4. ? IL-1 rec inhibitor anakinra in more severe cases

Front 70

(AD) TRAPS: Stands for?

1. Underlying prob?

2. Clinical sx?

3. Tx?

Back 70

TNF-Rec Assoc Periodic Syndrome

1. TNFRSF1A gene (AD!) - cysteine misfold of TNF rec

2. weeks of fever, serositis, arthritis, migratory rash, periorbital edema (also in CANDLE), renal amyloidosis

3. NSAIDs, corticosteroids, etanercept (but note TNF-mab may worsen!), IL-1 rec inhibitor anakinra

Hint 70

Think sx are TRAPPED for weeks!

Front 71

DIRA: Stands for?

1. Underlying prob?

2. Clinical sx?

3. Tx?

Back 71

Defic of IL-1 Rec Antagonist (anakinra)

1. basically anakinra deficiency

2. looks like NOMID with BONE issues (osteomyelitis, periosteitis), skin pustulosis

3. IL-1 rec inhibitor anakinra

Front 72

CANDLE: Stands for?

1. Underlying prob?

2. Clinical sx?

3. Tx?

Back 72

Chronic Atypical Neutrophilic Dermatosis with Lipodystrophy and Elevated temp

1. inactivating mutation of proteosome, leading to IFN type 1 production!

2. violet periorbital edema (also in TRAPS), lipodystrophy, panniculitis, FTT, synovitis

3. ?JAK kinase inhibitor (?tofacitunib)

Recall, Bortezomib also (-) proteosome

Front 73

DADA2: Stands for?

1. mutation? of course ADA2 def

2. Clinical sx?

3. Tx?

Back 73

Deficiency of ADA2

1. CECR1 (AR) mutation, leading to ADA2 deficiency

2. fevers, lacunar strokes!, livedo rash, necrotic lesions (recall also in LAD1), polyarthritis nodosa, mild (usu IgM) deficiency

3. TNF blockers

Front 74

Decreased immune system is a/w of which vitamins and minerals?

Back 74

Vit A (NK), Vit C, iron, zinc, selenium

Front 75

Invariant chain:

Loaded on which MHC _?_ in which cellular compartment

Back 75

MCH II

in the ER (endoplasmic reticulum)

Front 76

NKT cells are best described as:

a. MHC I restricted

b. CD1d restricted

c. Very small lymphocytes

Back 76

b. NKT cells are large lymphocytes that recognized both CD1d and MHC1 (KIR rec). However, NKT are CD1d restricted and MHC 1 nonrestricted.

CD1 present lipids.

Front 77

Gut ILC perform which?

a. secrete mucus

b. repress PMN phagocytosis

c. recruit macrophages

d. induce defensing

Back 77

d. GUT ILC helps maintain gut integrity by inducing enterocyte secretion of mucus and antimicrobial peptides.

Front 78

Which of the following is on all B cells but disappear when becoming plasma cells?

a. IgD

b. IgM

c. CD19

d. CD20

e. CD21

Back 78

answer: c. CD19

Front 79

Which joins first in forming Ig?

a. DH-->JH

b. VH-->DH

c. Vk -->Jk

d. Vl --> Jl

Back 79

answer:

a. DH-->JH (DJ of Heavy chain)

Front 80

Describe Pro-B cell VS Pre-B cell VS immature B-cell?

Back 80

Pro-B: has CD19 and CD10

Pre-B: gains Ig(mu) (no lt chain)

Immature: Ig(mu)+light chain = IgM

Front 81

Which process unique to B cells allows them to enhance the specificity and affinity of their antigen receptors?

a. Allelic inclusion

b. class switching

b. receptor editing

d. somatic hypermutation

e. telomerase activation

Back 81

answer:

d. somatic hypermutation (AID/UNG, in dark zone of germinal center)

allelic inclusion: only Ig(mu) for 1 allele (mom or dad) can be expressed

Front 82

Which of the following is NOT involved in forming IgM?

a. AID

b. kappa light chain

c. RAG 1/2

d. surrogate light chain

e. TdT

Back 82

answer:

a. AID leads to somatic hypermutation, which helps the affinity maturation (V arm) as well as class switching (H arm) in dark zone of germinal center

Front 83

Causes of hyperIgM?

Inheritance?

Which causes opportunistic infections (i.e. PCP)?

Back 83

Opportunistic infections (think T-cell licensing):

XL: CD40L (CD154) deficiency

AR: CD40,

No opportunistic infections:

AR: AID, UNG deficiency

Other:

Schnitzler's urticaria (later in life),

Ataxia Telangiectasia,

Waldenstrom's Macroglob

Front 84

Functions of AID & UNG?

Do they function in the bone marrow?

Back 84

Mutations in:

AID deaminates C (Cytosine) to U (uracil).

UNG removes the U, leaving empty spot to add any nucleotide!

No, they happen in the periphery in dark zone of germinal center, helping with B cell class switch and affinity maturation.

Front 85

What is "junctional diversity" and which protein is involved?

Back 85

Junctional diversity: DNA variability due to TdT (terminal deoxynuleotidyl transferase) randomly adding nucleotides during the V(D)J recombination.

Front 86

What does "alternative splicing" allow for B cells?

Back 86

Allows for IgM to be membrane-bound or soluble.

Front 87

How does Bortezomib work?

Which cells does it preferentially affect?

Side effect?

Back 87

Blocks proteosome, so cells can destroy bad proteins and gets choked up, undergoing autophagy and apoptosis.

(Recall CANDLE = proteosome issue)

Affects plasma cells.

Neuropathy (30%), low neutrophils/platelets, Shingles herpes zoster

Front 88

Immunodeficiency with enteropathy/ watery diarrhea?

Angry inflammatory bowel and bloody diarrhea?

Back 88

watery: CVID, IPEX, CTLA-4 (CD 152 talon)

angry: IL-10R deficiency, CGD, NEMO

Front 89

Pemphigus vulgaris:

ethnic group?

drugs?

describe.

Back 89

Jews, Mediterranean

-thiol (captopril, penicillamine)

Vulgar language (oral mouth blisters)

They are also despos (DesPo - DesMo - Antibodies against desmoglein 1[skin] or

desmoglein 3[muc memb])

Vulgar people are shallow and superficial (Superficial flaccid blisters above the basal layer, and will rupture easily. (Nikolsky's sign positive)

They are trapped in their own net. (Net-like IgG on immunoflorescence)

And die. (Patients die without treatment, poor prognosis)

And have tombstones on their graves. (Tombstone appearance of basal layer)

Front 90

Bullous pemphigoid, main antigen?

Back 90

BP Ag2 (causes blisters)

BP Ag1

Front 91

what is entopy?

Back 91

local production of IgE

Front 92

are nasal steroids helpful in pregnancy-induced rhinitis?

Back 92

no

Front 93

What causes ANTERIOR vs POSTERIOR subcapsular cataracts?

Back 93

anterior: AKC: atopic keratoconj

posterior: corticosteroids

Front 94

Which eye disease cause upper eyelid papillae?

Back 94

VKC (teens, photophobia, perilimbal dots) larger tarsal hypertrophy than GPC (contact lenses)

Front 95

sight-threatening eye conditions and differences?

other?

Back 95

VKC: vernal keratoconj --> boys, cobblestoning papillae, Horner-Trantas peri-limbal dots, severe photophobia, upper lid papille

AKC: atopic keratoconj --> teens to adults, itching, anterior cataracts, lower lid papillae, a/w atopic dermatitis

acute glaucoma

scleritis

iritis

uveitis

Herpes simplex keratitis

Front 96

Contact lenses can cause which conjunctivitis?

Back 96

GPC: giant papillary conj

Front 97

In asthma, which IL causes increased mucus production?

Back 97

IL-9, which also helps IL-4 mediated IgE class switching.

Front 98

What is in asthmatic sputum?

Back 98

a. Curshmann's spirals (excess mucus production)

b. Creola bodies (clumps of epithelial cells)

c. Charcot-Leyden crystals of eosinophilic proteins

Front 99

Sensitization to which mold is a/w persistent asthma?

Back 99

sensitization to Alternaria

Front 100

What does the Asthma Predictive Index API predict?

Criteria?

Back 100

Children < 3y/o with 4+ wheezing episodes in the past year, is likely to have persistent wheezing if:

One of:

- Parent asthma

- physician diagnosed AD

- aeroallergen sensitzation

AND 2 of:

- food sensitization

- >4% eosinophils

- wheezing apart from colds

Hint 100

API 1+2

Front 101

NHLBI 2007 cutoffs for persistent (mild vs. severe) asthma for child 4 y/o vs 5 y/o in regards to monthly nighttime symptoms?

Back 101

Mild persistent night sx:

4 y/o: 1/mo

5 y/o, 3/mo

Severe persistent night sx:

4 y/o: 1/wk

5 y/o: 1/night

Front 102

NHLBI, for 5 y/o, what is difference for monthly nighttime symptoms for assess SEVERITY vs CONTROL?

Back 102

For initial severity, >3/month is mild persistent, often nightly is severe persistent. (vs 4 y/o, where >1/month is persistent)

For control, 2/month is uncontrolled. (2/week is severe)

Front 103

ACT score for uncontrolled vs severely uncontrolled?

Back 103

ACT 16-19 is uncontrolled, 15 or less is severely uncontrolled

Front 104

Of the 6 steps for asthma treatment:

1. which step is low dose vs high dose ICS vs oral steroids?

2. what additional tx can you consider for step 2 in kids 5 year and older?

Back 104

1. Low dose ICS (step 2), high dose (step 5), oral steroids (step 6)

2. In kids 5 and older, you can consider SCIT or theophylline.

Front 105

What is Frey's syndrome? aka?

Back 105

aka auriculotemporal syndrome

face flush/sweat with flamey (spicy), flavored food

Front 106

Egg is in which:

1. vaccines?

2. sedative?

Back 106

Yellow fever vaccine

Rabies vaccine

(influenza)

Propofol

Hint 106

Yellow Rabies Egg

Front 107

Oral Allergy syndrome is a/w:

1. which peanut component?

2. which soy component? (tree?)

Back 107

peanut ara h 8

gly m 3 & 4 (birch pollen)

Front 108

which soy component is a/w asthma?

Back 108

gly m 1 & 2

Front 109

What is another name for milk protein intolerance in babies with bloody stools?

Back 109

FPIP (Food Protein Induced Proctocolitis)

Front 110

What is Heiner's hypersensitivity?

Back 110

milk hypersensitivity in kids, causing PNAs, IDA, FTT

Front 111

Which foods are a/w exercise-induced anaphylaxis?

Back 111

Celery

Wheat (omega 5 gliadin)

Shellfish / Fish

Fruit

Milk

Front 112

Which foods has oral allergy to this pollen?

Back 112

Ragweed:

Bananas & Gourd family (honeydew, watermelon, cantaloupe, zucchini, and cucumber)

Front 113

Mugwort has oral allergy to which foods

Back 113

celery & spices (like birch)

broccoli family

garlic/onion

bell/black pepper

Hint 113

think celery-mugwort-spice syndrome

Front 114

Birch pollen (looks like tri-nipples) is related to which foods?

Back 114

Birch (betula)

celery and spices (like mugwort)

fruits

soy (gly m 3/4),

PN (ara h 8)

hazelnut

Front 115

Which cytokines / receptors are a/w hypotension during anaphylaxis?

Back 115

Tryptase

Histamine

C3a

IL-6

TNF receptor 1

Front 116

What may serum Total:Mature tryptase ratio >20 vs <10 suggest?

Back 116

>20 means Systemic Mastocytosis

<10 other causes (i.e. acute anaphylaxis)

Note: beta tryptase not elevated in food-induced anaphylaxis (but may see 1.2x +2).

Front 117

What contaminant in heparin may cause an anaphylactic picture without skin symptoms?

Back 117

chondroitin sulfate

Front 118

Specific latex allergen a/w:

1. spina bifida

2. health care workers

3. foods

Back 118

1. hev b 1,3

2. hev b 6,5,7

3.

hev b 2: BO bell pepper, olives

hev b 6: BAC banana, avocado, chestnut

hev b 5: K kiwi

hev b 7: PT potato, tomato

hev b 13: P potato

Front 119

Venom IT is indicated for who?

When to continue VIT forever?

Back 119

Anyone with associated anaphylaxis

>16 y/o with systemic cutaneous (hives or angioedema)

SEVERE reaction,

systemic rxn on VIT,

honeybee allergy

?if skin or sIgE don't decrease

Front 120

Schnitzler syndrome

symptoms? when in life?

labs?

tx?

Back 120

autoinflammatory dz presents later in life with:

- non-pruritc neutrophilic urticaria

- periodic fevers

- arthraglia

a/w elevated IgM!

tx with anti-IL-1 (anakinra)

Front 121

What is emphysema, types, and causes?

Back 121

Emphysema is loss of elastic tissue in terminal airspaces.

Centrilobular (proximal acinar): upper lobes, cigarettes

Panlobular (panacinar): lower lobs, A1AT deficiency

Paraseptal (distal acinar): periphery, spontaneous PTX in young adult

Front 122

HAT vs HDACs

Back 122

HAT (Histone AcetylTransferase) is INFLAMMATORY by unwinding DNA to express NFkB and AP-1.

HDACs (Histone DeACetylases) is recruited by Corticosteroids and does the opposite, hence anti-inflamm.

Front 123

What is the difference between phototoxic and photoallergic?

Name some causative agents.

Back 123

Toxic (no prior sens):

foods (lime, celery, parsnip),

tar,

psoralens, furocoumarins,

tetracyclines,sulfonamines,

quinine (parasite)

amiodarone,

Allergic (requires prior sens):

chlorhexidine,

PABA,

thiourea,

sulfonylureas (T2DM)

dapsone,

quinidine(rhythm),

Both:

NSAIDs

diuretics

Front 124

Test for nickel contact dermatitis?

Back 124

DiMethylGlyoxime test (pink = positive)

Hint 124

Nicole goes to DMG to test Nickel.

Pink = passing positive

Front 125

Contact derm

Where are chromates found?

Back 125

leather and wet cement

Front 126

Contact derm

Where is cobalt found?

Back 126

in dental implants and artificial joints, also engines/rockets

recall: acrylates in dental & adhesive for occupational asthma

Hint 126

Kobe Bryant has cobalt teeth and rocket engines.

Front 127

Contact derm

What is myroxylon pereirae? what is it found in?

Back 127

Balsam of Peru

perfumes

cinnamon and vanilla

Front 128

most common cause of hand eczema in flower workers?

Back 128

AlstroEmeria (Peruvian lily)

Front 129

Sensitizing substances for most plants are mostly found in which fractions?

Back 129

oily oleoresin, but some in water-soluble glucosides.

Front 130

Preservatives are classified into which 2 main groups?

Name some examples of the releasers.

Back 130

Formaldehyde releasers:

Quaternium-15,

BNP (bromo-nitro-propane)

DMDM hydantoin,

-Linidinyl urea,

QLHB

Non-donors:

benzalkonium,

methylisothiazolinone,

para-ben,

phenoxy-ethanol,

PCMX/PCMC,

thimerosol

Front 131

What contact dermatitis agents are in hair products?

Back 131

cocamidopropyl betaine

glycerol thiolycolate

paraPhenyleneDiamine - think hair dressers & henna tattoo

(recall persulfate for occupational asthma)

Hint 131

Hair: cocoa butter, glycerin sugar,

Hair/henna: parafin wax

purposeful hair bleach

Front 132

What contact derm agents are in nail products?

Back 132

Acrylates (i.e. ethylacrylate)

Front 133

What is the main substance causing Toxidendron dermatitis?

There is cross-reactive to which fruit peels?

Back 133

UruShiol

mango peels

Front 134

Name 2 physical (non-chemical) sunblocking agents

Back 134

Titanium dioxide, zinc oxide

Front 135

Which steroids are use to patch test?

Name the 4 classes of steroids.

Back 135

A: hydrocort (test tixocortol)

B: triam (test budesonide)

C: betametasone

D: hydrocort-17-butyrate

Front 136

What contact derm agent is common in topical creams?

Back 136

EthyleneDiamine

Hint 136

Think Ethyl's skin cream

Paraphen for her hair

Front 137

What rash can sometimes precede HAE attacks?

In what other conditions can you see it?

Back 137

erythema marginatum, also in RA

Front 138

HAE treatments:

1. acute PRN?

2. prophylaxis for life?

3. before procedures?

4. for pregnancy?

Back 138

1. C1 (Berinert, Ruconest) ecallantide, icatibant

2. Cinryze, androgens, FFP (chronic and forever)

3. Berinert, androgens (before anesthesia)

4. plasma-derived C1 inh (Cinryze, Berinert)

Front 139

ddx for T- SCID?

Back 139

CD3 (e,d,g,e)

CD45

CD25 nail (IL-2Ra), also IPEX-like

CD127 tunic (IL-7Ra, also rec for TSLP)

Hint 139

Think TCR & T cells!

Front 140

ddx for Bare Lymph Syndrome, CD8 vs CD4 lymphopenia?

Back 140

CD8: deficiency of ZAP70, TAP, tapasin,

CD4: mutations (MHC2, RFX5, FRXAP, FRXANK), UNG119 & LCk deficiency, HIV inf, XLP

Hint 140

8 zap tap tap

4 bare HIV lyck their X-lip

Front 141

XLP-1

mutation? which encodes?

function of that protein?

Back 141

XLP-1: SH2-D1a mutation, encoding SAP

SAP links SLAM (with its ITSM) to Fyn/CD3

SAP = SLAM-Associated Protein

SLAM = Signaling lymph activation molecule

Front 142

Immunodeficiency with severe IBD?

Back 142

deficiency of IL-10

recall, severe diarrhea also in CGD & NEMO

watery: CVID, IPEX, CTLA4 (CD 152 talon)

Front 143

Name some live vaccines

Back 143

Adenovirus

Nasal Influenza, MMR, Varicella/Zoster, Yellow Fever, oral Polio, Rotavirus, BCG, Typhoid, Vaccinia (smallpox),

Front 144

What virus is contraindicated with intussussception?

Back 144

Rotavirus

Front 145

Persons with isolated B cell immunodeficiency may receive which vaccine?

Back 145

varicella

Front 146

Gelatin is in which vaccines

Back 146

Hep B,

influenza,

MMRV,

Varicella/HZ,

Yellow Fever

Hint 146

Jello HIMVY

Front 147

Neomycin is in which vaccines

Back 147

MMR, varicella/VZ, Polio

Hint 147

Neo Polo MV

Front 148

Egg allergy & vaccines: guidelines?

Back 148

If hives, PCP give vaccine, watch 30 minute

If systemic rxn, give Flublock (RIV3) if adult, otherwise allergist give and wait 30 min.

Front 149

Name the IL-12/IL-23 blocker that can be used for psoriasis

Back 149

ustekinumab

watch out for mycobacterium inf

recall, IL-12 deficiency = MSMD

Front 150

PDE-4 blocker for psoriasis?

PDE-4 blocker for COPD? SE

Benefits

Back 150

psoriasis: Apremilast

COPD: Roflumilast, GI side effects (n, v, wt loss)

anti-inflammatory

Recall, PDE-5 inh is Viagra

Front 151

IL-17 blocker

Back 151

secukinumab

Front 152

Hemidesmosomal antigens in Bullous Pemphigoid?

Back 152

Autoantigens against:

BP Ag1

BP Ag2 (BP 180)

Think elderly, rigid deep blisters (negative Nikolsky), hemidesmosomes on basement membrane

Hint 152

Think pemphig OLD

Front 153

symptoms in pemphigus folicaceous

antigen

Back 153

crusted scaly skin (subcorneal acantholysis)

ab against Desmoglein1 on skin (vs 1 or 3 in pemph vulgaris)

Front 154

IgA skin deposition:

DH vs. Linear IgA Dermatosis.

Causative agents?

Treatment?

Back 154

DH: gluten

Linear IgA Derm: vancomycin!! & other drugs

TX: dapsone (since neutrophil mediated), topical steroids.

Also, gluten-free diet with DH.

Front 155

Example of dominant negative dz

Back 155

STAT3 JOB

Front 156

Example of haploinsufficiency

Back 156

GATA-2 haploinsufficiency

(recall, low NK bright, mono/mac)

CTLA-4 haploinsufficiency

(CD 152 talon)

Front 157

Basophil Activation Test (BAT) lead to surface expression of which markers?

Back 157

CD203c (usu. on surface at low levels)

CD63 (coats histamine secretory granules)

Front 158

What is the receptor for Stem Cell Factor for mast cell activation?

Most common activating mutation?

Back 158

CD117 (c-kit rec)

D816V mutation

Front 159

The common progenitor for MC, basophils, and eosinophils has which CD marker?

Back 159

CD34

Front 160

which receptor on Mast cells binds vancomycin, opiods, and icatibant?

Back 160

MRG-X2 ("murg X2")

Front 161

Receptor for C5a

CD number?

Expressed on which cells?

Back 161

CD88, on MCtc (mast cells) & basophils

Recall, C5a is a powerful chemotactic factor for neutrophils.

Front 162

Criteria for systemic Mastocytosis? (Major & minor)

Back 162

1 M + 1 m OR 3 m

Major: dense infil >15MC/aggre

minor:

- >25% spindle shaped

- D816V

- CD2 or CD25 nail (IL2Ra) on MC

- tryptase > 20ng/ml

Front 163

3 criteria for MCAS (mast cell activation syndrome)?

Back 163

1. classic sx in 2 organ system

2. increase mast cell marker (tryptase, urine metabolites) with sx

3. response to anti-mediator therapy

Front 164

which ANCA (cytoplasmic proteins in neutrophils) are a/w which disease?

Name examples of ANCAs.

Back 164

c-ANCA (PR-3): GPA, EGPA (less common), (ANCA by indirect immunofluorescent, confirmed by PR-3 by ELISA)

p-ANCA (MPO): EGPA (more common), Microscopic PA

Front 165

Cryoglobulinemia vasculitis in which diseases?

lab findings

Back 165

infection (esp Hep C!!!)

neoplasms

connective tissue dz

(+) cyroglobulin, (+) RF, (+) hep C, low complements

Front 166

vasculitic urticaria vs. regular urticaria?

Back 166

painful, last >24 hours, less pruritic, central red, leave hyperpigment +/- systemic features

Front 167

What is Felty's syndrome?

Back 167

RA

splenomegaly

neutropenia

Hint 167

Felty's Rhematoid Arthritis triad

Front 168

Name the CTLA-4 (CD152 talon) blocker?

Back 168

abatacept

Front 169

Name the IL-6 Rec blocker (used in RA)

Back 169

Tocilizumab

Hint 169

6 toucans liz zoo

Front 170

Name a JAK inhibitor

Back 170

tofacitunib

Hint 170

jack tofu nibbles

Front 171

Highly specific auto-ab for SLE Lupus?

Back 171

anti-DNA, anti-Sm

Front 172

Auto-ab in Neonatal SLE, cutaneous SLE & Sjogren syndrome

Back 172

Anti-Ro(SSA) and anti-La (SSB)

Recall, T cell infiltration in Sjogren biopsies.

Front 173

Auto-ab in Systemic vs Cutaneous Drug-induced SLE

Back 173

systemic: anti-histone

cutaneous: anti-Ro/La

Front 174

Main tx in Rheumatoid Arthritis vs SLE?

Back 174

RA: methotrexate or other DMARDs +/- biologics

SLE: hydroxychloroquine

Front 175

Auto-ab in scleroderma

(aka systemic sclerosis), including CREST)

Back 175

Anti-centromere

Anti-toposiomerase (SCL70)

Anti-RNA polymerase 3

Recall, use ACE-I instead of steroids!

Front 176

Which Rheum dz do you avoid corticosteroids due to SE of renal crisis?

TX instead?

Back 176

scleroderma (systemic sclerosis), use ACE-inhibitors instead!

Front 177

HIV diagnosis, which antibody & antigen test(s) are good for:

1. Screening Rule OUT?

2. Confirmatory Rule IN?

Back 177

Rule OUT:

1. ab: ELISA, Rapid HIV, Western

2. antigen: p24 (4th gen screen)

Rule IN:

1. ab: Western blot or Indirect IF

2. antigen: PCR HIV DNA or RNA

Front 178

Which HIV protease inhibitor Med can increased inhaled or nasal CS to systemic levels?!

Back 178

Ritonavir

Ritonavir puts steroids Right On INTO your body!

Front 179

HIV prophy organisms & meds?

Back 179

PJP & Toxo: Bactrim

Histo: itraconazole

Cocci: fluconazole

MAC: macrolide

Front 180

In EOE, which cytokines are involved?

Back 180

IL-5

IL-13 recruit eotaxin-3 (CCL26)

Front 181

how much DM is recommended in SCIT?

Airborne?

Back 181

7 micrograms per injection

No, quickly settles (large 10-35 micron)

Front 182

Most common cat allergen? Airborne?

Back 182

Fel d 1 (homologous with uteroglobulin)

minor: Fed d 2-4

Yes, <10 micron so airborne for hours

Front 183

Most common mouse allergen

Back 183

Mus m 1 (urinary protein)

Front 184

Primary, sec, and tert prevention of allergic dz

Back 184

Primary: prevent sensitization

Secondary: prevent allergic dz

Tertiary: prevent dz severity

Front 185

Level of DM to induce sensitization in most studies, VS nonallergic subjects?

Back 185

Most: 2-10 mcg/ gm air

nonallergic: 20 mcg/g

Front 186

How long after cat removal to reduce allergen levels to background level of <10 mcg/g?

Back 186

10-20 weeks (3-4 months)

Front 187

For HES (Hypereosinophilic Syndrome),

1. MC genetic mutation for myeloproliferative variant? tx?

2. tx for non-myeloprol?

Back 187

1. 4q12 del (FIP1L1-PDGFRA fusion), tx is imatinib TK inhibitor

2. glucocorticoids (but r/o out Strongyloides first)

Front 188

Where can imatinib bind, and give examples?

Back 188

1. abl (i.e BCR-abl philadelphia chromsome)

2. c-kit/CD117 (non-D816V)

3. PDGR-r (i.e. FIP1L1-PDGRa)

Front 189

Two-tier vs single-step testing for Lyme/borrelia?

Back 189

Two: ELISA --> Western

Single: ELISA measuring IgG for Variable major protein-like sequence-expressed (VIsE) 6th invariant region (C6)

Front 190

When must I use penicillin desens for syphyllis/Treponema?

Back 190

PCN-allergic patients in:

- neurosyphyllis

- pregnant women

- congenital syphyllis

Front 191

Adult pneumonia (CAP) first vs second line treatment?

Back 191

doxycycline or macrolide (azithromycin, clarithromycin)

2nd line: fluoroquinolone OR (1st line + beta lactam)

Front 192

collarette flakes on eyelids are c/w?

Back 192

seborrhea of the eye

Front 193

Name 6 eye drops meds that are dual anti-histamine / mast-cell stabilizers

Back 193

OAK ABE

olopatadine

alcaftadine

ketotifen

azelastine

bepotastine

epinastine

Front 194

Differentiate btw the Type 4 hypersensitivity in regards to:

- reactions / sx

- cell types

- cytokines

Back 194

4A: contact derm = macrophages, TH1 = IFN-g

4B: DRESS, maculopap = eos, TH2 = IL-4, IL-5

4C: SJS/TENS, drug hep = CD4, CD8 = CTL, perforin, granzyme

4D: Pustular (AGEP) = PMNs, T-cell = IL-8

Hint 194

A. Contact your date. Get your mac on and interfere G.

B. Get DRESSed maculopapular, EzPHIL brings the IL4,5 bling.

C. SJS at 10s, where you do drugs and get CD8 to shred.

D. Pus oozing at the afterparty with neutrophils cleaning up on Aisle (IL) 8.

Front 195

Describe the 3 models of Drug Allergy mechanisms of action..

Back 195

1. Hapten (med) binds to self-proteins to engage TCR.

2. P-I. Pharm (med) engaged TCR directly.

3. APR. Med alters peptide repetoire (binding specficity), creating neoepitopes that can engage TCR.

Front 196

Drug allergy: dapsone

HLA, ethnic?

reaction?

Back 196

HLA B13:01 13 Asians

"dapsone hypersens syn"

Front 197

Drug allergy: carbamazepine

1. HLAs? & Ethnic groups?

2. Drug reaction?

Back 197

HLA B15:02 (15 Han Chinese)

HLA A31:01 (31 A Europeans)

Stevens Johnson (type 4C, CD8, CTL)

Front 198

Drug allergy:

HIV RTI med? HLA? ethnic group?

Back 198

Abacavir

HLA-B57:01 57 white HIV,

delayed hypersensitivity

Front 199

Drug allergy: allopurinol

HLA, ethnic group

reaction?

Back 199

HLA B58:01 58 Asians

severe cutaneous reactions

Front 200

PCN MAJOR vs minor determinants

Back 200

MAJOR: Pre-Pen (Benzyloyl-Polylysine)

minor: pen g, -loate

Hint 200

MAJORly PREtty BENZ LOYAL!

pen g minor are low apes.

Front 201

MC drug causing serum sickness (F, arthralgia, LN, proteinura) in children?

why?

Back 201

cefaclor

toxic metabolites from altered metabolism

Front 202

causes of drug-induced thrombocytopenia?

other ab in TTP?

Back 202

- Heparin (HIT): IgG to heparin-platelet factor (HPF 4)

- Vancomycin: ??

- Quinine-dep glycoproteins 2b/3a, 1b/9

anti-ADAMTS13 (von willebrand cleaving factor) in TTP

Front 203

Agents causing PIE (pulmonary infilatrates with eosinophilia)?

Back 203

Nitrofurantoin & other abx

NSAIDs

gran lungs = MTX

eos + lungs = Nitro, NSAIDs

eos + nose = AERD NSAID

eos + kidney = ceph, pcn

Front 204

agent causing acute granulomatous ILD?

Back 204

methotrexate

eos + lungs (PIE) = Nitro, NSAIDs

Front 205

In Toxic Epidermal Necrolysis, should you use steroids?

Back 205

NO! It's contraindicated!

Front 206

This monobactam has the same R chain as this cephalosporin.

Back 206

AZtreonam & CefTAZadime

Front 207

Chemotherapy agents that can cause IgE-mediated allergy & sensitization after multiple rounds of that treatment?

Back 207

Platinum compounds (cisplatin, etc.)

Front 208

In AERD patients, which cytokines are:

1. Increased?

2. Decreased?

Back 208

1. LTC4 synthase, LTs, & LT receptors (cysLTR 1 & 2)

2. Decreased lipoxin & EP2 (rec for PGE2, which (-) 5LO)

Front 209

1. What does prostaglandin PGE2 block?

2. What is its receptor that is decreased in AERD?

Back 209

1. PGE2 blocks 5-LO

2. Receptor is EP2

Recall, zileuton also block 5-LO

Front 210

Which type of reactions to one particular NSAID is NOT going to cross-react to other NSAIDs?

Back 210

anaphylaxis

hypersens pneumonitis

aseptic meningitis

Front 211

Cytokine Release Syndrome is a/w which cytokines and mab?

Back 211

IL-6 & TNF-a

rituximab (anti-CD20)

muromunab (anti-CD3)

Front 212

Btw DRESS & TEN, which has more autoimmunity vs mortality?

Back 212

TENS: more mortality

DRESS: more auto-immunity (think 4B. eos IL-4,5)

Front 213

Cold urticarias with Negative Ice-cube test?

Back 213

1. Cholinergic (cold-induced) urt

2. Systemic cold urticaria

3. Cold-dependent dermatographism

4. PLAID (AD) - PLYg2

5. FCAS (AD) - CIAS1-AP

Front 214

What condition has been associated with solar urticaria?

Back 214

Erythropoietic protoporphyria

Front 215

Name physical urticarias that can be passively transferred by serum.

Name known Ig associated.

Back 215

Cold-induced (IgG,M,E, cryoglob)

Solar Type 1 & 4

Dermatographism (IgE)

Front 216

auto-antibodies aw CIU?

Back 216

thryoid: anti-TPO > anti-TG

IgG,IgM against α chain of FCeR1 (high affinity rec), and possibly augmented by C5a

Recall, C5a rec is CD88 on MCtc & basophils and is a powerful chemotactic factor for neutrophils.

Recall, Langerhan/DCs lack beta unit on FCeR1

Front 217

decreased C1q and anti-C1q antibodies is seen in which diseases?

Back 217

AAE (acquired angiodema)

HUV (hypocomplementic urticaria vasculitis)

Front 218

Factor I does what?

Deficiency may lead to what?

Back 218

Factor I inhibits C3

Factor I deficiency may lead to urticaria

Without Factor I blocking, C3 is overactivated, leading to lots of C3a anaphylotoxin and urticaria.

Front 219

Where does C1-INH block?

mutation gene?

Back 219

C1-INH blocks C1, pre-kallikrein, and Factor 12.

Mutation: SERPING1 (AD)

Front 220

Which HAE tx has risks for anaphylaxis?

Back 220

ecallantide (Kalbitor)

Front 221

MC cause of chronic urticaria?

Back 221

physical urticaria - 20%

(the rest (80%) is idiopathic CIU)

Front 222

which physicial urticaria have similar sized hives as aquagenic urticaria?

Back 222

Cholinergic & Aquagenic both have very fine (1-3 mm) hives.

Front 223

Which HAE tx almost always have local injection reactions?

due to which receptor?

Back 223

icatibant (Firazyr) - skin on fire!

MRG-X2 (recall, opiod rec on mast cells)

Front 224

What is RA,NTES?

Other name?

Role?

Back 224

a chemokine (Regulated on Activation, Normal T cell Expressed and Secreted)

CCL5

(+) NK cells & also suppress HIV

Front 225

Hypersensitivity Pneumonitis (HP) predominant with CD4 or CD8?

What cell may you seen on histo lung bx?

Back 225

Trick question!

Early: CD8

Later: CD4, neutrophils

multinucleated giant cells

Front 226

Agent in Farmer's lung HSP?

Back 226

Thermophillic actinomyces

Mycopolyspora faeni

Hint 226

Front 227

Woodworker agents causing HSP?

Which also cause OA?

Back 227

Alternaria mold

Suberosis in corkwood

Plicatic acid in wood (also in OA)

Hint 227

Woodwork with:

alternate clubs (made from wood) in hand

driving cork Subaru

pliable wood in the trunk

Front 228

Normal sweat chloride levels

Back 228

30-40 mmol/L

60 is positive!

Front 229

What does A1AT inhibit?

Genotypes? i.e. M, etc.

type of emphysema?

Back 229

A1AT (-) Neutrophil elastase

M (normal) -> S -> Z -> Null (worse)

panlobular/panacinar

(vs centrilobular in cigarettes, paraseptal in ptx)

Front 230

Most important radiographic finding in ABPA?

Back 230

central bronchiectasis

+/- fleeting infiltrates

Front 231

Anemophilous?

Entomophilous?

Amphiphilous?

Back 231

Anemophilous: wind pollinated Entomophilous insect pollinated Amphiphilous: BOTH

Front 232

Which fungi are prevalent on prevalent on Dry, Windy Days?

Back 232

Dry, Windy Days

Alterneria (club shaped)

Cladosporum (cigar shaped)

Also Epicoccum

Hint 232

Smoking cigar with club in hand on hot windy day

Front 233

Which fungi are prevalent on Humid (steamy) Nights?

Back 233

Ascospore

Basidiospore (mushrooms, puffballs, smuts, rusts)

Hint 233

Busy Smuts and Ass on Humid Nights

Front 234

Virus with molecular mimicry to:

glutamic decarboxylase (GAD)?

Back 234

Coxsackie B

Front 235

Virus with molecular mimicry to:

MBB (major basic protein)

Back 235

Hep B

Hint 235

Hep B in Major League Baseball player

Front 236

Virus with molecular mimicry to:

acetylCHOLine receptor?

Back 236

Herpes simplex

Front 237

Name antigens on RBCs

Back 237

Rh

D, Kell, Duffy

A,B, O

H-antigen is in O group and is absence in "Bombay" phenotype in LAD2 subjects

I antigen (IgM, cold-reactive AIHA)

?? mult sites (IgG, warm-reactive AIHA)

P antigen (IgG, paroxysmal cold hemoglobinuria, positive polar ice cube test)

Front 238

What tests should you do with insect-sting anaphylaxis?

Back 238

skin test --> sIgE --> repeat skin test 3-6 months later

(If tx with VIT, treat all positives tests regardless if no sting before).

tryptase (positive >11)! (for systemic mastocytosis)

Front 239

Risk factors for severe venom/sting reactions?

Back 239

older

male

repeated stings

honeybee stings

severity at initial sting

baseline tryptase

beta blockers / ACE-I

Front 240

Most common indoor molds?

Back 240

Penicillium

Aspergillus

Front 241

Which aeroallergen IT is standardized in the US?

Who oversees it?

Back 241

Grasses

Short ragweed

DM

Cat

CBER under FDA

Front 242

With Immunotherapy (IT), what markers goes up?

Back 242

specific IgE increased initially, then decrease later.

IgG1 (early), IgG4 (later)

TH1: IFN-g (IgG2 class switch), IL-12

Treg: IL-10, TGF-b (recall, these also stimulate IgA class switch IL-10 also helps IgG1, IgG3 class switch.)

Front 243

With immunotherapy (IT), what receptor (& CD name) is decreased?

With IT, which cytokines are decreased?

Back 243

low affinity FCeR2 (CD23)

decreased HAF, PAF, TNF

Front 244

Immunotherapy is indicated for AD with sensitization to what?

Back 244

DM

Front 245

WHat med should you consider in refractory anaphylaxis due to beta blocker?

Back 245

glucagon (has inotropic & chronotropic effects on the heart, since not dependent on adrenergic receptors)

Front 246

Consider stopping which meds to start IT?

Back 246

beta-blockers

??ACE-I (for VIT)

Front 247

All of these AIT forms are efficacious EXCEPT for:

1. epicutaneous (sticker)

2. intranasal

3. intralymphatic

4. Rinkel

Back 247

Rinkel (initial dose based on serial intradermal)

Front 248

Name some adjuvants for immunotherapy (IT).

Back 248

Center-Al - aqueous, alum-precipitated, less systemic reactions

MPL (Monophosphoryl Lipid A)

Front 249

General rule of thumb in future prognosis of stopping immunotherapy?

Back 249

The longer, the better.

After 3-4 years of therapy, 30-40% of patients report return of symptoms 3-4 years later.

Front 250

FDA Pregnancy Risk categories?

Back 250

A. no risk

B. no risk in humans (budesonide, montelukast, cromolyn, Xolair)

C. ?

D. Positive risk

E. Contraindicated!

Front 251

Main allergen types in plant/paleo diet (seeds, nuts, legumes)?

How about fruits?

Back 251

prolamin (includes lipid transfer protein in fruits)

cupin

Hint 251

Lame cupids eat paleo diet.

Front 252

Beta-agonist: What makes LABA longer acting?

Back 252

They have long hydrophobic tails that is stuck to theplasma membrane “exo site”, whereas the “albuterol” portion is hydrophilic.

Front 253

Which inhaled beta agonists are full vs partial agonists?

Back 253

Formoterol: Full agonist, Fast-acting

salmeterol& albuterol are partial agonists.

Front 254

Name some adverse effects of beta agonists?

O2, e+/-, & glucose?

Back 254

Tremor for the first week

MI, Prolonged QT, palp/arrhythmia

Transient dip in oxygen, K+, Mg+ and increase in glucose

Front 255

How does beta-2 desensitization affect the medication?

Back 255

It decreases duration(i.e. 8 hrs instead of 12 hrs), NOT degreeof bronchodilation. It also does NOT affect albuterol response in acute asthma.

Front 256

Name some ANTI-cholinergics inhalers.

Which musc receptors do they bind?

Back 256

SAMA:

Ipratropium (M2 = M3)

LAMA:

Tiotropium (M3 > M2, dissociates from M2 quicker)

Umeclidinium

Front 257

Which of the 5 muscarinic/acetylcholine receptors are predominant in lungs?

Their action in the lung?

Back 257

M3: the main one that causes bronchoconstriction!

M2: excitatory & inhibitory (inhibition may cause paradoxic bronchoconstriction)

M1

Front 258

theophylline MoA & SE?

Back 258

(-) PDEphosphoDiesterase, which usu breaksdown cAMP/cGMP.

Theophylline = Increased cAMP/cGMP = bronchodilation, anti-inflammation.

SE: (-) adenosine receptor, causing (+) CNS, arrhythmias, gastric secretions

Front 259

Theophylline therapeutic range

Back 259

10-25 mg/L

Front 260

What decreases theophylline? Remember, theo has no effect <10mg/L.

Back 260

Meds: rifampin, AEDs

Smoking

Children

High protein, low carb diet

Front 261

What increases & "boosts" theophylline? Recall, toxicity >25mg/L.

Back 261

Meds: OCPs, Zileuton, macrolide, cipro, cimetidine, verapamil, SRI

Liver disease, CHF, alcohol

Viral disease, PNA, vaccines

Old age

Front 262

Which meds decreases steroids?

Back 262

Increased metabolism:

rifampin,

AEDs

antacids (reduce bioavailability)

Front 263

Which meds increases steroids?

Back 263

Decreased metabolism:

ketoconazole,OCPs

Methylpred also affected by clarithromycin and troleandomycin.

Front 264

Things that are Inflammatory:

Back 264

IL-1, IL-6, TNF

IL-12, IL-18, IFN-g

HAT

PDE

Front 265

Things that are ANTI-inflammatory:

Back 265

Steroids

NSAID

H2 receptor (increase IL-10)

IL-10 / TGF-beta (IgA class switch)

IL-35

HDAC

PGE2 (-) 5LO

Front 266

Antihistamine MoA?

Back 266

Inverse agonist: binds H rec, and pushes it to the inactive form.

Front 267

AntiHistamines non-H1 effects? In which meds?

Back 267

Anti-CHOLinergic: all 1st gen

Alpha-blocker: promethazine

Local anesthetic: diphenhydramine

Anti-serotonin: cyproheptadine (appetite)

Front 268

Important details of glucocorticoid structure:

Back 268

A-ring C1-2: double bond increases GLUCO > mineralocorticoid activity)

C-ring C11:pro-drug ketone (C=O) must be converted to OH group to be active

i.e.cortisone => hydrocortisone, pred => prednisolone

D-ring: C16,17,21:additions increase potency and enhance degradation.

Front 269

Cortisol is bound to which plasma proteins?

Back 269

Transcortin (high affinity, few around)

Albumin (low affinity, lots around)

Front 270

glucocorticoid receptors?

Which one is active?

Back 270

GR-alpha (active)

GR-beta (inactive) – increased in steroid resistance, fatal & nocturnal asthma, nasal polyposis

Front 271

glucocorticoid pathway of mechanism of action?

Back 271

GC goes inside cell and bind GR-alpha, causing heat shock proteins to dissociate, allowing GR-a to become phosphorylated dimer to go to nucleus to bind DNA:

1. TRANS-ACTIVATE (binds GRE on anti-inflamm genes: IL-10,GILZ, MKP-1, IkBa, TTP [degrades inflamm mRNA]), HDAC [re-wraps histone])

a. Note, GC may (+) HDAC at low conc and may (+) HAT (for anti-infl genes) at high conc.

2. TRANS-REPRESS (binds and inhibits TF that promotes inflammatory genes).

Front 272

Name other SE of steroids (skin, mm).

Back 272

Skin (striae, acne, hirsutism)

MSK (aseptic necrosis, myopathy)

Local: dysphonia, thrush

Front 273

Effects of LTB4 vs cysLT (LTC/D/E)?

Back 273

LTB4: chemoattractant for Neut & Eos (Note: LTB-R1 is high affinity.)

cysLT: POTENT bronchoconstriction >>> histamine.Also recruits some Eos. (Note: cysLT-R1binds LTD > C > E and is blocked by meds).

Hint 273

LT Body 4 is ATTRACTIVE for Neut & Eos

cyst LT C,D,E chokes

Front 274

In AD, decrease in which tight junction protein may allow Langerhan to reach out outside?

Back 274

Claudin-1 (CLDN1 gene)

Front 275

Filaggrin:

1. Odd Ratio for PN, AD, & asthma?

2. Found in lungs?

3. Downregulated by which cytokines in severe AD?

Back 275

1. PN 5x, AD 3x, and asthma 1.5x

2. No, not found on bronchial epithelium

3. Filaggrin is downregulated by IL-4, IL-13.

Front 276

TSLP

1. Expressed by which cells?

2. Does what downstream?

Back 276

1. Keratinocytes expressed TSLP

2. TSLP (+) DCs to release chemokines CCL17 and CCL22, which attract T cells. TSLP also (+) naïve T => TH2.

Recall:

CCL26 (eotaxin-3)

CCL27 (CTACK)

Front 277

In AD, infiltrating T cells release which ligand for E-selectin (CD62E)?

Back 277

CLA (Cutaneous Lymphocyte-associated Antigen)

(Recall, selectin CD62 also bind CD15 Sialyl Lewis X/A carb as well, and its fucosylation is the problem in LAD2.)

Hint 277

T cells send out cute LALAs (CLA) to be selected to E-selectin

Front 278

Cytokines found acute vs chronic AD?

IFN-g does what to keratinocytes?

Back 278

Acute AD (TH2): IL-4, IL-5

Chronic AD (TH1): IL-13, IL-12, IFN-g

Keratinocytes=>TSLP, (+)DCs

IDEC (+) Tcell to make IFN-g

IFN-g (+) Fas (CD95) on keratinocytes, ??? though decreased IFN-g systemically

Front 279

Chemokines in Atopic Dermatitis?

Back 279

CCL5 (RANTES)

CCL11 (Eotaxin)

CCL13 (MCP-4)

CCL17 (TARC)

CCL27 (CTACK)

IL-16 (attracts TH2)

CTACK = cutan T cell attact chemokine, attracts CLA(+)/CCR10(+) T cells

TARC = thymus & activ.-reg chemokine

Recall, CCL17 & 22 also attract T cells.

CCL26 is eotaxin 3.

Front 280

Which skin DCs:

1. has more FCeR1? and (+) Tcells to make what?

2. has FCeR1 missing which subunit?

3. has Birbeck granules?

Back 280

1. IDECs > Langerhans

IDEC (+) T cell to make IFN-g

2. Langerhans lacks classic beta subunit of FCeR1

3. Langerhan has Birbeck gran

recall, autologous ab to alpha unit of FCeR1 in CIU

Front 281

In AD, increased microbes & infections is due to what?

Back 281

Increased TH2, leading to decreased antimicrobial expression (i.e. H-beta defensin 2 & 3, Human cathelicidin LL-37) by keratinocytes.

Front 282

In nasal provocation challenge, which are Pre-formed vs Newly-formed mediators in MC & basophils degranulation?

Back 282

Preformed: histamine, tryptase, bradykinin

New: CT, PGD2

Front 283

In nasal provocation, do steroids decrease secretions of:

- albumin (plasma leak)?

- glandular secretions?

Back 283

- albumin (plasma leak)? yes

- glandular secretions? No

Front 284

Risk of AR?

- Maternal smoke

- High grass pollen count

- Mediterranean diet

Back 284

- Maternal smoke: increased risk

- High grass pollen count: DECREASED risk (thinkfarm/rural setting)

- Mediterranean diet: DECREASED risk (thinkanti-oxidants)

Front 285

Does IntraNasal CS affect airway BHR?

Is there BHR in NARES?

Back 285

YES! INS decreased BHR.

Yes, there is BHR in NARES.

Front 286

For AR, does skin test or sIgE have better sensitivity?

Back 286

Neither, both have ~70% sensitivity

Front 287

Gustatory rhinitis:

- Due to which fibers?

- Treatment?

Back 287

Due to (+) of sensory c-fibers by capsaicin on TRPV1receptor

TX: ipratropium

Front 288

rhinitis of pregnancy:

1. Do INS steroids work?

2. Frequency? When resolves?

Back 288

1. Yes, if they had pre-existing AR. But NO if it’s purely pregnancy-induced. Instead, use nasal decongestant & supportivemeasures. (Avoid ORAL decongestion, which is a/w gastroschisis! Yikes!)

2. 20%. Resolves within 2 weeks of delivery.

Front 289

Atropic rhinitis (think old women with halitosis):

1. Which bacteria implicated?

2. TX?

Back 289

1. Klebsiella ozaenae

2. TX: lavage, debride crust

Hint 289

think ozone crust

Front 290

DDX for perforated nasal septum?

Back 290

Cocaine

GPA (wegener’s)

Front 291

Meds a/w rhinitis

Back 291

NSAIDs

OCPs

Anti-HTN

PDE-5 inhibitor (sildenafil, etc)

Pyschotropics & gabapentin

Recall, PDE-4 inh is Roflumilast & Apremilast

Front 292

Orchard & Timothy grass oral allergy is cross-reactive to which foods?

Back 292

orchard grass:

melon, PN, potato/tomato (like latex hev b 7)

timothy grass:

chards, orange

Front 293

When do sinuses develop?

Back 293

ME at birth

F by Five yrs of age

S by Six (really 8-10)

Hint 293

(Think up and back, or alphabetically.)

Front 294

Where do the sinuses drain?

Back 294

M, E (anterior), F => ostiomeatal complex below middleturb

E (posterior) => superior meatus

S => sphenoethmoid recess above superior turb

Front 295

What is concha bullosa?

Haller cell?

Mucocele?

Back 295

Concha: Air in middle turbinate (Associated with septal deviation, but NOT sinusitis!)

Haller cell: Ethmoidal air cells along orbit floor (It DOES increase risk of acute sinusitis.)

mucocele: Epithelial lined sac completely filling the sinus. Mostly in frontal or ethmoid sinus, a/w CRS and may expand. Removed with surgery.

Front 296

Acute rhinosinusitis:

1. duration definition?

2. associated receptor?

Viral vs Bacterial sinusitis?

Back 296

4-12 weeks (3 months)

ICAM (CD 54) is rec for both integrin & rhinovirus

Viral if <10 days & not worsening

Bacterial if worsening(or double worsening) within 10 days, or no improvement after 10 days

Front 297

CRSwith Polyps (W v E) vs No polyps:

Which is TH2 or TH1?

Which one may respond to oral steroids?

Back 297

POLYPS: Western polyps & AERD, think TH2 & eosinophils, but Asian polyps and CF have more TH1/TH17 and neutrophils!!

No polyps: TH1 & IFN-g

Oral steroids (as well as saline rinses & biologics) can help shrink polyps!

Front 298

Which main fungi is associated in AFS (acute fungal sinusitis)?

(FYI, AFS is kinda like ABPA for the nose.)

Back 298

Aspergillus /Alternaria

Bipolaris

Curvularia

Drechslera

Exserohilium

Fusarium

Helminothosporium

Hint 298

ABCDEF,H

Front 299

How to test for CSF leak?

Back 299

Beta 2 transferrin (from CSF)

Front 300

Which allergen HIGH exposure is most associated with asthma morbidity?

Back 300

Cockroach

Front 301

What diet can increase or decrease FENO?

Back 301

INCREASE: viral URI, AR

sandwich meats, veggies (spinach, green been, carrots, beets)

Decrease: alcohol, smoking

(other: spirometry, CF, pulm HTN)

Front 302

Significance of periostin?

Back 302

Reflect IL-13 stimulation

Suggests TH2 phenotype & response to glucocorticoids, along with FENO & serum eos

Front 303

milk allergen

Back 303

Bos d 4-8

Hint 303

Bossy milk

Front 304

egg allergen

Back 304

gal d 1-5

Hint 304

Egg gal

Front 305

shrimp allergens

Back 305

Lit v 1 (myosin)

Pen a 1 (tropomyosin)

Recall, per pen der (tropomyosins)

Front 306

fish allergen

Back 306

gad c 1 (parvalbumin)

Front 307

Hazelnut allergen

Back 307

Cor a 8,9,14

Front 308

Apple allergen

Back 308

Mal d 1,2

Front 309

Food allergies clinical cross-reactivity %

Back 309

Cow/goal milk 92%

Melon/fruits 92%

SF 50%

Fish 50%

TN 35% (tho skin test 90%)

Pollen/peach 50%

Latex/fruit 35%

Milk/beef 10%

Legumes 5%

Front 310

Risk factors for PN allergy?

Risk factor for food allergy

Back 310

PN: family member wtih PN allergy (Increase 7-fold!!), severe eczema, egg allergy

Food allergy: family history of atopic dermatitis and atopy.

Front 311

FPIES foods?

Back 311

MC: milk, soy

Older kids: egg, wheat (& rice, oats), nuts, fish, poultry

Hint 311

Think classic 6 + grains & poultry

Front 312

PPV, & 50% NNV for ImmunoCap for Food allergy:

1. egg

2. milk

3. PN

4. fish

Back 312

sIgE (kUA/L)

egg 7 (2 if 2 y/o), NNV 2

milk 15 (5 if 1 y/o), NNV 2

PN 14, NNV 2 w/ hx (5 if no hx)

Fish 20

Hint 312

egg 7 (2), 2

milk 15 (5), 2

PN 14, 2 (5)

Fish 20

Front 313

Which component allergens of peanut & hazelnut have been a/w clinical severity?

Back 313

peanut: ara h 1, 2, 3

hznut: cor a 9, 14

Front 314

Which food additive can trigger asthma?

also consider sensitivity to which food colors?

Back 314

sulfites (though RARE)

red: carmine

red-orange: annatto

Front 315

What is chemosis?

Back 315

edema of conjunctiva

Front 316

Name the inhibitory Fc receptor that has cytoplasmic ITIM? CD name?

Back 316

FCgR2b (think b=block!), aka CD32

FCgR2a is activating

Front 317

Mold associated with winemaker's HP?

Back 317

Botrytis

Hint 317

Think winemaker's bottles

Front 318

Contact derm allergen in henna

Back 318

paraPhenyleneDiamine

hair dressers & henna tattoo

Front 319

Do Quaternium-15 and BNP (bromo-nitro-propane) release formaldehyde?

Back 319

Yes, these preservatives do.

Front 320

What is Stachybotrys?

Back 320

indoor black mold

Front 321

Dz with these ANA patterns:

1. homogenous?

2. speckled

3. nucleolar

4. centromere (think anti-centromere ab)

Back 321

1. SLE lupus

2. (everything)

3. scleroderma, polymyositis

4. scleroderma

Front 322

DRESS is a/w which infection?

Back 322

HHV-6

Front 323

HEPA filters down to what size

Back 323

0.3 microns

Front 324

What is the most potent TLR ligand for B cell proliferation?

Back 324

CpG (ligand for TLR 9)

Front 325

Cytokines involved in pathway of steroid-induced apoptosis of eosinophils?

Back 325

Caspase-3

NJK

Fas (CD95)

Front 326

Cytokines involved with isoswitching to IgG (subclass), IgA, & IgE?

Back 326

IgG1/3: IL-10

IgG2: IFN-g

IgG4 & IgE: IL-4, IL-13

IgA: IL-10 & TGF-b

Front 327

Which HAE tx contraindicated with rabbit allergy?

Back 327

recomb C1 inh (Ruconest)

Front 328

What marker may be elevated in cryoglobulinemia?

Back 328

RF (IgM)

Front 329

BAL is predominantly CD4 or CD8 in:

1. HP

2. Sarcoidosis

Back 329

HP: early is CD8 (restrictive), late is CD4/PMN (mixed restrictive/obstructive)

Sarcoidosis: CD4

Front 330

False (+) VDRL in which condition?

Back 330

Anti-phospholipid syndrome

Front 331

Which IgE receptor is low-affinity?

CD name?

what happens with allergy IT?

Back 331

FCeR2 (CD23)

decrease with IT

Front 332

Which histamine receptor is Anti-inflammatory?

How?

Back 332

H2 receptors

by decreasing chemotaxis, increase ANT-inflamm IL-10

Front 333

Which CD markers are receptors for viruses?

Back 333

CD21: EBV, HHV8

CD46: adenovirus

CD54: rhinovirus

CD48: NK rec for EBV-infected B cells

Hint 333

21 Nut

46 Rush

48 Rave

54 Law

Front 334

Cytokines important for T cell survival?

Back 334

IL-7 for memory T cell, B cell dev

IL-15 for memory CD8 (and NK stimulation!)

Front 335

What downregulates vs upregulates IgE receptors (FcER1 & FcER2/CD23)?

Back 335

ACTIVATING:

Signal 1: IL-4, IL-13, IL-9

"Signal 2": CD40L, APRIL/BAFF, TLR-ligand

DOWNREGULATING:

Xolair, IFN-g,

IL-21 (bound to FcER2) --> TF Bcl-6 (THf), SOCS

Front 336

IgE receptors subunits on FCeR1 vs FCeR2?

CIU has ab to which subunit?

Which cells lack beta unit of R1?

Back 336

FcER1 is either tetramer (a,b,g,g) or trimer (a,g,g with no beta).

FcER2 is only trimer.

In CIU, there is ab to alpha unit of FcER1.

DC (incl Langerhans) lack classic beta unit in FcER1.

Front 337

IgE receptor FcER1 vs. FcER2:

Which is high vs lower affinity?

Which rec is downregulated by Xolair?

Which downregulate IgE by membrane form when bound to CD21?

What cleaves it to soluble forms to upregulate IgE?

Back 337

FcER1: high affinity; downreg with Xolair

FcER2 (CD23): lower affinity, membrane binding to CD21 downregulates IgE. Membrane stalk can be cleaved to soluble CD23 by ADAM or Der p 1, and upregulates IgE.

CD23a on B cells,

CD23b on other cells.

Front 338

Elevated IL-6 may present with what symptoms?

In which diseases?

Back 338

fever, microcytic anemia, low albumin, high CRP

Castleman's lymph hyperplasia

Atrial myxoma

Multiple myeloma of plasma cells

Front 339

Lymph node hyperplasia:

1. Kikuchi disease?

2. Kimura disease?

3. Castleman?

Back 339

1. necrotizing LAD, self-limited

2. cervical LAD with elevated IgE

3. autoinflammation-like (fever, anemia, elevated CRP) due to IL-6 overproduction.

Front 340

Name ligands for:

1. TLR 1, 2, 6

2. TLR 5

3. mouse TLR 4

4. TLR 7,8

5. TLR 9

6. TLR 3

Back 340

TLR 1, 2, 6: lipoprotein, peptidoglycan

TLR 5: flagellan

mouse TLR 4: LPS

TLR 10: influenza

TLR 7,8: ssRNA

TLR 9: CpG DNA (most potent for B cell stimulation)

TLR 3: dsRNA

Front 341

In thymic development, which mutation is a/w:

Nude SCID (hairless)?

Back 341

FOXN1 TF mutation

Front 342

In thymic development, which TF is a/w:

DiGeorge 22q deletion

Back 342

Tbx1 (Tbox) TF mutation

Front 343

What cell surface marker is indicative of "exhaustion"?

Back 343

PD1 (programmed death)

Front 344

What what age to we convert to more CD45RO > CD45RA?

Back 344

~college age

Front 345

CLR C-Lectin family (extracell):

1. What does dectin 1 & 2 bind?

2. DC sign binds?

Back 345

1. fungal beta glucan, & Der p 1, (+) TH17

2. sugars mannose, fucose

Front 346

NK KIR receptors:

1. Binds what?

2. Which receptor subtype is activating or inhibitory?

KIR = Killer-cell Ig-like Receptors

Back 346

KIR receptors bind MHC I on infected cell.

KIR short tail is stimulating

i.e. KIR2DS1

KIR long tail is bLocking and has 2 ITIMs each. i.e. KIR2DL1

Front 347

NK memory cells are triggered by what?

Back 347

Infections & Nickel hypersensitivity.

(But we don't know of any specific receptors for it.)

Recall, IL-15 helps memory CD8 but also stimulates NK cell differentiation.

Front 348

common beta chain:

- CD name?

- receptor family includes?

Back 348

CD131 donut

receptors for IL-3, IL-5, GM-CSF

(Recall: CD132 demon is common gamma chain)

Front 349

TNF-alpha

- produced by which cells?

- cut by what to soluble trimer?

- a/w syndrome

- a/w sign during anaphylaxis?

- up or down with IT?

- TNF-alpha receptor blockers used for which anti-inflammatory dz?

Back 349

TNF-a is produced by mast cells (pre-formed), TH1, among others

TNF-alpha (and TNF rec 1) are both cut by TACE (TNF-alpha converting enzyme) from membrane trimer to a soluble trimer. (TNF rec 2 is a monomer.)

TNF is aw Cytokine Release Syndrome and Hypotension in anaphylaxis.

TNF is decreased with IT.

TNF-alpha blockers used for TRAPs (only etanercept) and DADA2.

Front 350

Fever, increased CRP and Ig, low albumin is indicative of which response?

Associated cytokines?

Back 350

Acute phase reaction

IL-6

Front 351

Intracellular PRRs (pattern recog rec):

1. NLR (+) what cytokines?

2. NLRP3 binds what? associated with which gene mutation?

3. NLPR1 binds what?

Back 351

1. IL-1, IL-18

2. Alum in vaccines

CAPS (CIAS1-Associated Periodic Syndrome)

3. bacterial muramyldipeptide

Front 352

What does STING stand for?

What does it (+)?

STING GoF mutation is a/w which syndrome?

Back 352

STimulator of INterferon Gene

Type 1 interferons

SAVI syndrome (STING-Associated Vasculopathy with onset in Infancy)

Front 353

MC factor for IVIG side effects in PIDD patient?

Back 353

concurrent infection

Front 354

In making IVIG, what removes prions?

Back 354

nanofiltration

Front 355

In making IVIG, what inactivates lipid envelope viruses?

Name those viruses

Back 355

S/D & Caprylate removes

HIV, Hep B & C

Front 356

3 autoinflammatory disease a/w aMyloidosis proteins?

Back 356

CAPS (FCAS, MW, NOMID/CINCA)

FMF

TRAPS

Front 357

What is pathergy?

Seen in which disease?

How is Koebner phenomenon different?

Back 357

Exaggerated skin injury after minor injury.

seen in Behcet's

Koebner has lines

Front 358

Intestinal lymphagiectasia with low albumin, low Ig, and low WBCs likely has preferential loss of which cells?

Back 358

CD4 cells

Front 359

Treatment for Wegener's GPA renal disease?

Avoid which medication?

Back 359

steroid,

cyclophosphamide or retuximab

AVOID MTX (bad for kidneys)!

Hint 359

Return the cycling steroids to those Wagon kidneys.

Front 360

Rheumatoid Arthritis:

extra-articular sx?

Back 360

Pulm (ILD, org pna, pleural eff, nodules)

CV: Vasculitis, Pericarditis

Skin, Eyes

Felty's splnmegaly/neutropenia

Front 361

Which pollen can release breathable particles laden with PolyGALACTURONase allergen?

Back 361

Timothy grass (phleum)

Front 362

Honeybee and Vespids share limited cross-reactivity which which standardized allergen?

Back 362

hyaluronidase

Front 363

Which lab do you use to check for cross-reactivity from sensitization to multiple venoms?

Back 363

inhibition immunoassay

Front 364

Risk factor for Idiopathic anaphylaxis?

Back 364

atopy

Front 365

Mutation that is a risk factor for insect venom anaphylaxis?

Back 365

mutation in Tyrosine Kinase Growth receptor (TKGR)

Front 366

What vital sign is affected maximally by both H1 & H2 receptors?

Back 366

pulse pressure

Front 367

Nasal provocation, which cytokines are in the:

Early response?

Late response?

Back 367

Early: histamine, bradykinin, PGD2

Late: IL4/5/13, eotaxin, ECP

Front 368

Which are Kinase vs Phosphatase:

SH1, CD45, calcineurin, and SHP1/SHIP?

Back 368

kinase: includes lck, syk, SH1

phosphatase:

(+) includes CD45, calcineurin

(-) includes SHP1, SHP2, SHIP

Front 369

Do ITAM, ITIM, and adaptor proteins have intrinsic kinetic activity?

Name some adaptors.

Back 369

No, they do NOT have intrinsic kinase or phosphatase activity!

Adaptors: LAT, SLP-76, SLP-65(BLNK), Grb-2

Front 370

Where does lck kinase play a role?

Back 370

lck is near the cytoplasmic tail of CD4 or CD8 and phosphorylates ITAMs on the zeta chains and well as recruit ZAP-70.

Front 371

Which kinase (+) Phospholipase C-gamma in T cell vs B cell?

What does PLC-g do?

PLC-g mutation in which dz?

Back 371

T-cell: iTK

B-cell: BTK

PLC-g catalyzes PIP2 into DAG and IP3!!!

PLAID syndrome

Front 372

What phosphorylates PIP2 into PIP3? mutation dz?

PIP3's role?

Back 372

Pi3 kinase mutation can cause CVID

PIP3 recruits iTK (recruits PLC-g) and aKT (inhibits Bcl-2/Bcl-XL apoptosis & activates mTor pathway of cell growth)

Front 373

IP3 (IP man) stimulates which ER sensor, to open up which channel?

What happens downstream?

Back 373

IP3 (+) STIM1 sensor, causing CRAC channel to open the floodgates of calcium into the cell.

Calcium-bound CALMODULIN activates the phosphatase CALCINEURIN, which frees NFAT.

Front 374

DAG (+) PKC, which (+) which complex?

Think Dagger in your Pocket

Back 374

CBM

- CARD 11 (prev CARMA1)

- Bcl 10

- MALT 1

Recall,

Bcl-2, Bcl-XL in apoptosis

Bcl-6 (STAT3 in THf)

Front 375

Ras MAP kinase is activated by which 2 GEFs (guanine exchange factor)?

Back 375

Ras is (+) by:

RasGRP, recruited by DAG

SOS (Sons of Seven), recruited by LAT/Grb-2

Front 376

Name cell type, other TF, and cytokines for:

STAT1

Back 376

STAT 1=> macrophage => IL-12

Front 377

Name cell type, other TF, and cytokines for:

STAT3? related to 2 cells

Back 377

STAT 3=> THf (Bcl-6) => IL-21

STAT 3=> TH17 (RorgT) => IL-17

STAT3 mutation = JOB HIES

Recall:

Bcl-2, Bcl-XL in apoptosis

Bcl-6 (STAT3 in THf)

Bcl-10 in CBM complex -> NF-kB

Front 378

Name cell type, other TF, and cytokines for:

STAT4?

Back 378

STAT 4=> TH1 (Tbet) => IL-12, IFN-γ, TNF-α

Front 379

Name cell type, other TF, and cytokines for:

STAT5?

Back 379

STAT 5=> Treg (Foxp3) => IL10, IL-35, TGF-b

Front 380

Name cell type, other TF, and cytokines for:

STAT6?

Back 380

STAT 6=> TH2 (GATA 3) => IL-4/13

Front 381

ISHAM diagnosis criteria for ABPA

Back 381

Asthma/CF

positive Aspergillus skin test

IgE > 1000 IU/ml (less if other criteria present)

2 of the following:

# serum precipitating ab Aspergillus,

# radiograph findings,

# AEC >500/mcl

Front 382

Immunodeficiencies with susceptibility to HSV encephalitis?

Which one is AD vs AR?

What foreign particle does it recognize?

Back 382

AD: TLR 3 deficiency (independent of MyD88 & IRAK-4). TLR3 binds dsRNA (or synthetic Poly I:C in research).

AR: UNC93b

Front 383

amorphic mutations in this XL disease causes incontinentia Pigmenti in females and lethal in males?

Back 383

NEMO (NFkB Essential MOdulator) deficiency

Front 384

Deficiency in DAF and/or CD59 results in what?

Back 384

Paroxysmal nocturnal hemaglobinuria (night dark urine)

CD59 is anti-hemolysis

Front 385

Source of HSP in humidifier & sauna? (Think air)

Back 385

Aureobasidium

Front 386

Source of HSP with malt worker?

Back 386

Aspergillus

Hint 386

Asperger kid drinking malt

Front 387

Which agents are a/w Occupational Asthma:

1. HMW>10kd, usu IgE-mediated?

2. LMW <10kD:

- spray paint, plastics, insulation?

- paint, adhesives?

- nail salon, dental?

- hair bleach?

- wood & sawdust?

- welder?

- solder?

- cleaning agents?

Back 387

1. HMW (foods)

2. LMW includes:

- isocyanates (spray paint, plastics, insulation)

- anhydrides (paint, adhesives)

- acrylates (nail salon, dental)

- persulfate salts (hair bleach)

- plicatic acid (cedar)

- platinum salt (welder)

- colophony (cosmetics, wax; made from pine tree)

- aldehydes, quaternary ammoniums (cleaning agents)

Hint 387

Cyan sprays, plastics, insulation

A hybrid paint

Acrylic nails & dental implants

Purposeful bleached hair with coco, glyc, parafin

Pliable wood

Platinum welder

Colorful cosmetic wax

All the quarter cleaning agents

Front 388

What type of proteins are responsible for Oral Pollen Syndrome?

Back 388

Profilins

Front 389

Which Ig can activate complement?

alternative pathway???

Back 389

classic: IgG1/3

alternative: IgG3/4, IgA1/2

Front 390

Which IgG subclass have shortest half lives?

Back 390

IgG3, 8-9 days whereas IgG1/2/4 are 23 days.

Front 391

Ig effector function, can kill though which two ways? which Ig are involved?

Back 391

ADCC: (No IgM!), IgG1/2/3/4.

Complement: IgM, IgG 1/-/3/4

Front 392

IL-2 receptor consists of 3 forms with which combinations of subunits?

Recall, disease association(s)?

Back 392

High affinity:

IL2Rb,

common gamma demon (CD132),

IL2Ra nail (CD25)

Intermediate affinity:

common g demon (CD132),

IL2Ra nail (CD25)

low affinity:

only IL2Ra nail (CD25)

Recall:

1. CD25+CD2 found in mast cell dz.

1. CD25 deficiency in T- SCID & IPEX-like dz.

2. NOT included in IL-15 Receptor, which share IL2Rb & CD132 (common g), but has its own IL-15Ra.

Front 393

What is lymphotoxin (LT)? Coded by which genetic region?

The 2 forms consist of which units? Which is soluble vs membrane bound? receptors?

membrane form's role is important for what?

Role of membrane form?

Back 393

Lymphotoxin (LT) is TNF-beta, coded by MHC class 3 region.

soluble: LTa3, alpha trimer, rec is TNF rec 1 or 2

membrane: LTa1b2 (1 alpha + 2 beta); rec is LT beta receptor (LTbR); important for fetal development of lymphoid tissue!

Front 394

Which IL-1 & TNF receptor is activating?

Back 394

Activating: IL-1 receptor 1 and TNF receptor 1

Nonactivating: IL-1 receptor 2 is decoy and TNF receptor 2

Front 395

what does IL-18 do?

Back 395

aka "IFN-gamma inducing factor"

think TH1 response: IL-12, IL-18, IFN-gamma

Front 396

Which cytokines help B cells to class switch to IgA?

Back 396

IL-10 & TGF-beta

(recall, these are T-reg cytokines that are generally "anti-inflammatory")

Front 397

APRIL and BAFF can both bind to which 2 receptors for B cell activation?

Back 397

BCMA & TACI

BCMA binds APRIL more strongly!

Front 398

What are decoy chemokine receptors named?

Which is used by Plasmodium in malaria?

other function?

Back 398

Atypical ChemoKine Receptor

ACKR1-4

ACKR1 (DARC) in malaria; mutation = resistance

Besides decoy, can transport a chemokine from one side of cell to other.

Front 399

Chemokine receptors are which type of receptor molecule?

Back 399

G-protein (7 transmembrane portions)

Front 400

Integrins (a/b):

- have what CD nomenclature?

- binds which ligands?

Back 400

Integrins (i.e. a1b1, LFA, Mac) CD11/18, CD49/CD29

Bind Ig family molecules:

ICAM-1 liar (CD54), rhinovirus

ICAM-2 dozen (CD102)

ICAM-3 lose (CD50)

ICAM-4 Noreen CD242

Front 401

Selectins
- has which CD nomenclature?

- binds which ligands (CD names too)?

Back 401

Selectins are CD62E/P/L

Bind Sialyl Lewis X (CD15) and CLA

Recall, CD15s problem in LAD2

Front 402

What other proteins are coded in MHC Class I, II, & III gene regions?

Back 402

MCH I gene: MHC I

MHC II gene: MHC II + TAP (MHC I presentation)

MHC III gene: Complement, Lymphotoxin (TNF-b)

Front 403

IRAK-4 deficiency lead to which types of infections?

Back 403

pyogenic infections.

MyD88 pathway (recall, girl with pcv meningitis)

Front 404

What is C3 Nephritic Factor cause disease?

Back 404

stabilize C3bBb so Factor H can't even degrade it, so complement keeps activating

Front 405

What helps transport IgA & IgM into the lumen?

Back 405

Poly-Ig receptor

Front 406

Which cytokines (+) class switch to IgA?

Back 406

TGF-beta, IL-5

Front 407

Chemokines in T-cell zone (parafollicular) & its receptor?

Back 407

CCL19/21 binds to CCR7

Front 408

Pro-Apoptosis vs Anti-apoptosis pathways?

Back 408

Pro-apoptosis:

Intrinsic: Bim/Bax/Bak => caspase 9

Extrinsin: Fas (CD95): FasL (CD178) => caspase 8

Caspase 8/9 => Caspase 3

Anti:

Intrinsic: Bcl-2, Bcl-XL

Recall:

Bcl-6 (STAT3 in THf)

Bcl-10 in CBM complex -> DAG/NF-kB

Front 409

Which CD marker is on monocytes & part of TLR4?

Back 409

CD14

Front 410

plasmacytoid DC has which receptor (also CD name?) but lacks which other CD marker found in other DCs?

Role of plasmacytoid DC?

Back 410

Has IL-3 receptor (CD123 denim), but lacks CD11 integrin

P. DC secretes IFN-a, helping viral infections!

Front 411

Which chemokine receptor is a/w susc to West Nile virus?

Back 411

CCR5, the same one that confers resistance HIV

Recall: CCR5 binds CCL2/3

CCR7 binds CCLL 19/21

Front 412

Vit B12 is elevated in which diseases?

Back 412

ALPS

Myeloproliferative HES

Front 413

Tranplant risks: lowest to highest?

matched sibling, no conditioning

matched cord

matched unrelated

haploidentical

Back 413

Lower risk:

Matched sibling

Matched cord & unrelated

haploidentical

Highest risk

Front 414

Describe graft rejection:

1. Hyperacute rejection

2. Acute

3. Chronic

GVH:

1. Acute

2. chronic

Back 414

1. rejection on table (preexisting ab)

2. days to months; T-cell killing

3. months to years; CD4 vasculopathy

1. GVH acute macpap rash <100 days

2. GVH chronic skin/alopecia/myositis/FTT/cytopenia/pericarditis >100 days

Front 415

In transplant, what is conditioning for?

When to avoid conditioning?

Example reduced regimen?

When is it unnecessary?

Back 415

Conditioning reduces host's immunity to make "space" for new B cells.

Avoid/reduce conditioning with active infections.

Ex. reduced regimen: fludarabine, alemtuzamab, busulfan

Unnecessary for SCID with matched sibling donor.

Front 416

Risk for which infections with TNF inhibitors?

Back 416

mycobacterium TB reactivation

Fungal (i.e. histo)

Front 417

which is best med to stop mast cell degranulation?

cromolyn, cetirizine, epi, steroid

Back 417

epinephrine (b2 agonist)

Front 418

Choose a specific human eosinophil marker:

arysulfatase, EDN, MBP1, MBP2

Back 418

human is MBP2

Front 419

Cathepsin G is unique to which cell?

Back 419

neutrophils, which also has LTB4

Front 420

Basement shower mold?

Back 420

Epicoccum nigrum

Front 421

DLCO is decreased in which obstructive airway disease?

Back 421

COPD (not asthma!) due to decreased surface area in alveoli.

Recall, DLCO decreased in restrictive lung diseases.

Front 422

Anaphylaxis in fish may be due to which parasite?

Back 422

Anisaki

Front 423

What is Bacillary angiomatosis?

Back 423

vascular nodules, fever, LAD in HIV (+) people with Bartonella

Front 424

How long after RBCs & IVIG can you give Live vs non-live vaccines

Back 424

Live: 3 months after blood products

Non-live: 6-8 months after

Front 425

Anti-CCP antibodies in which dz?

Back 425

RA rheumatoid arthritis

Front 426

What is hypervaccination?

Used for what?

example regimen?

Back 426

Lots of vaccination to drive up Ig in complement deficiencies

Recall, opsonins include Ig and complement.

Meningococcal vaccine every 3 years.

Front 427

Dz with early complement (C1-4) deficiencies?

Back 427

SLE (apoptotic cells)

atherosclerotic diseases

With deficiency, there is no complement to help clear apoptotic cells or cholesterol.

Front 428

Measure what to assess environmental load of:

1. Gram (+)

2. Gram (-)

Back 428

1. Gram Pos: muramic acid in the thick peptidoglycan cell wall (vs single layer in GN).

2. Gram Neg: endotoxin found in LPS outer membrane of GN

Front 429

Infections with NK deficiency?

Deficiency with low NK bright?

Deficiency with low NK dim?

Back 429

viruses, esp herpes

low NK bright (precursor): GATA2 deficiency

low NK dim (mature): MCM4 deficiency & perforin deficiency causing HLH

Front 430

NK cell (+) by which cytokine?

Back 430

IL-15 (which also helps CD8 survival)

Front 431

North vs South? Examples?

Pooideae

Panicoideae

Chloridoideae

Back 431

Northern:

Pooideae (Timothy, rye, blue, etc.)

Southern:

Panic: Bahia, Johnson, corn, sugarcane

Chlor: Bermuda, prairie