Flashcards in IMMUNO Deck (421):
CCL11 other name
CCL11 also known as eotaxin
what is the rhinovirus receptor on T cells
What are the major criteria for the API
parental asthma, sensitization of aeroallergen, Dr dx of AD
What are the minor criteria for the API
food sensitization, >4% Eos, wheezing apart from colds
What is considered a positive API
one major OR two minor criteria
Chemokines important in AD
CTACK (cutaneous T-lymphocyte attracting chemokine; CCL27, TARC (thymus and activation-regulated chemokine)
What does TSLP stand for?
TSLP: Thymic Stromal Lymphopoeitin
Which cells contain Birbeck Granules
Prolamin superfamily types of foods and proteins
seed storage proteins, lipid transfer protein, alpha-amylxase/protein inhibitors
7s vicillim and 11s globulins
PR10 protein family
Bet v 1, birch
Atopy leads to increased risk for which agents anayphlayxis?
LIER: latex, idiopathic, exercise, radio contrast (NOT meds)
Which agent does pre-treatment not help in anaphylaxis risk?
Which agents does pre-treatment HELP in anaphylaxis risk?
radiocontrast, cold and flueorescien-related anaphylaxis
thermophilic actinomyteces (sacchropolyspora rectivirgula, thermoactinomyces vulgaris) - moldy hay, grain, compost
Thermoactinomyceses sacchari; moldy sugar cane
Humidifier fever/AC lung
Thermoactinomyces (T. Vulgarin, T. sacchiri, T candiddus); Klebsiella, Acanthamoeba
Hot tub lung
Mycobacterium avian complex (MAC) or Cladosporium - from contaminated water
Basement shower lung
Epicoccum nigrum; mold growing in unventilated showers
Malt worker's lung
Cheese worker's lung
Penicillium casei - exposure to moldy cheese
Machine operators lung
Pseudomonas, Acinetorbacter, Mycobacter; PAM is a machine operator
Bird fancier's lung, pigeon breeder's lung
Avian proteins - exposure to droppings, feathers, serum
Detergent worker's lung
Alternaria - moldy wood dust
Botrytis cinerea - mold on grapes
Summer type HP
Trichosporium cutaneous - contaminated houses, coming in Japan
El niño lung
Pezizia - exposure to mushroom fungus after heavy rainfall
Candida albicans (Think Bill Clinton has a yeast infection)
Wheat weevil lung/miller's lung
Sitophilus - contaminated flour
Chemical worker's lung
TDI (toluene diisocyanate), MDI (diphenylmethane diisocyanate)
Epoxy Resin worker's lung
Plastic Worker's lung
in shampoos, facial cleaners and bath products; one of the MCC contact sensitivity
MCC cause of contact HSN in hair dressers
Permanent wave solution; one of the MCC contact sensitivity
What to avoid if sensitive
in topical creams; need to avoid nystatin, aminophylline, and piperazine antihistamines (meclizine and cyclizine)
Allergen in Henna
paraphenylenediamine (same as hair dresser contact derm)
Only long term/prophylaxis medication for HAE?
Cinryze: plasma derived C1-INH
(I can see the CINRYZE (sunrise) in the long run"
Which HAE medication has a risk for anaphylaxis:
Kalbitor (kallikrien inhibitor ecallantide) - "Kalli has anaphylaxis"
Live Vaccines Mnemonic
BOYs Love The CRiMe: BCG, OPV, Yellow Fever, Live, Typhoid, Chicken Pox (varicella), Rubella, MMR, Mumps
Which two sinuses have the most complications in sinusitis?
Which sinuses are most likely to have polyps?
Which allergen is CRS w polyps more likely to be sensitive to?
A type of CVID - KMT2D mutation
WHIM syndrome mutation
CXCR4 mutation AD
Where do neutrophil precursors arrest in severe congenital neutropenia
Molecular defect for SCN1
Molecular defect for SCN2
Molecular defect for SCN3
Molecular defect for SCN4
Molecular defect for SCN5
Which 2 micro bugs is CGD NOT associated with?
Streptococcus PNA, Pneumocystis (PJP) infections
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.)
CCL5 : aka / receptor
CCL5 aka RANTES -- receptor CCR 1, 3, 5
CCL11: aka / receptor
CCL11 aka Eotaxin -- receptor CCR3
CCL17: aka / receptor
CCL17 aka TARC-- receptor CCR4
(1+7 = 8 / 2 = 4)
CXCL8: aka / receptor
CXCL8 aka IL8 -- receptor CXCR1, 2
Spina bifida antigens for latex
Hev b1, Hev b 3
Where dies latex allergen come from?
Cytoplasmic exudate of the hevea brasiliensis tree, Hev b 1-13
Hev b 2 assoc food?
Bell pepper, olive
Hev b 5 assoc food?
kiwi, potato, sugar beet
Hev b 6 assoc food?
bananda, avocado, chestnut, sweet pepper
Hev b 7 assoc food?
Hev b 7 = "patatin like proteins"; potato and tomato
Hev b 13 assoc food?
MCC of biting insect systemic run?
Triatoma - painless bite
What do Asain Lady Beetle cross react with?
Asian lady beetle (Harmonia Axydris) - cross reactive with cockroach
Order of sinus development? Mnemonic
My Extremely Sweet Friend: Maxillary, Ethmoid, Sphenoid, Frontal
Sinus drainage - Mnemonic?
FM AM, PS SS
Frontal, Maxillary, Ant Ethmoid - Middle meatus
Post Ethmoids, Sphenoid - Sphenoethmoid recess=poabove Superior turbinate
What does the middle meatus open into?
Which mutation is seen in Myeloproliferative HES?
PDGFRB, FGFR1 rearrangements; Deletion of 4q12 --> FIP1L1-PDGFRA fusion
What is the mutation and inheritance in familial HES?
Well's syndrome is...
How do you treat myeloproliferative HES?
Imatinib: tyrosine kinase inhibitor
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
What staining pattern are mast cells identified with?
+ CD2 +/- +CD25, +CD117
What is the spirochetal infection causing Lyme disease?
What tick does Borrelia burgdorferi infect?
Rx for Lyme?
Doxy, cefuroxime, amoxicillin (>8 years old)
Human Monocyte Ehrlichiosis infectious cause?
Principle vector tick for Ehrlichiosis is?
Treatment for Ehrlichiosis?
Tetracycline and chlroamphenicol
Human Granulocyte Anaplasmosis infectious agent?
Anaplasma phagocytophilum is infectious agent
Human Granulocyte Anaplasmosis vector?
Ixodes scapulars is Human Granulocyte Anaplasmosis vector
Infectious agent causing babesiosis?
Babesiosis symptoms/lab findings
Babesiosis: fatigue, fever; hematologic and liver Abn
Is p-anca or c-anca more specific in Wegener's (granulomatosis w polyangiitis)?
Which vaccines are considered protein vaccines?
Tetanus and Diphtheria (T cell dependent)
Which vaccines are considered polysaccharide vaccines?
Meningococcal and Pneumococcal (Pneumovax); T cell independent
Which vaccines are considered polysaccharide vaccines?
Meningococcal and Pneumococcal (Pneumovax); T cell independent
Which type of Ag do NKT and gamma/delta T cells respond to?
The meningococcal and pnuemococcal vaccines are considered what type of vaccines?
What are the cytokines of MHC1
IFN alpha, beta, gamma
What are the cytokines of MHC2
Which components are part of the MHC1 processing pathway?
Calnexin, Calreticulin, Erp57, and tapasin are "chaperone proteins" in which MHC pathway?
Where on chromosome 6 are TAP proteins encoded?
Class 2 locus
Where on chromosome 6 are LMPs from the proteasome encoded?
Class 2 locus
What are components of the MHC2 processing pathway?
cathepsins, Ii (invariant chain), CLIP (class 2 invariant chain peptide), HLA-DM
What molecule removes CLIP for peptides to bind MHC2?
HLA-DM removed CLIP
MHC2 def: what is usually deficient?
transcription factors for MHC2: MHC2TA, RFX5, FRXAP, FRXANK
Types of infections in MHC class 1 def?
sinopulmonary, granulomatous skin lesions, necrobiosis lipoidica
Types of infections in MHC class 2 def?
diarrhea, hepatosplenomegaly, transaminitis, sclerosis cholangitis (Cyrpto); pulm infxn: PJP, encapsulated bacteria, herpes, RSV; meningitis
Treatment of MHC1 vs MHC 2?
MHC2: HSCT; MHC1: aggressive infection Rx
What is the inheritance of MHC 1+2?
What 2 factors determine lymphocyte tolerance?
Antigen concentration and Antigen affinity determine tolerance; high [ ] and affinity promote negative selection
What markers do Tregs express?
Tregs: CD4, CD25, FOXP3
What is the other name for CD25?
What cytokines do Tregs depend on?
IL2, TGFB; IL10 helps maintain tolerance
Which cytokines are released in super antigen stimulation?
IFNg and TNFa
What kind of cells express AIRE?
AIRE is expressed by thyme medullary epithelial cells
Where are Tregs made?
What cytokines assist in regulation of Tregs?
TNF family member, signal for B cell homeostasis
What enzyme is class switching mediated by?
Class switching is mediated by AID instead of RAG
How much Ig does the adult human produce in 1 day?
2-3 grams of Ig per day
Which Ig fixes complement the best?
IgM fixes complement the best
Which Ig has the highest total body concentration?
Which Ig has the most daily production?
Best Ig for ADCC?
IgG subtype with shortest half life?
IgA1 vs IgA2 in location?
IgA1: serum and respiratory tract
IgAs: lower GI tract
Ig with shortest half life?
IgE: 2 days
Class switch recombination changes which region: V vc C?
Class switch changes C region
Somatic hypermutation changes which region: V or C?
Somatic hypermutation changes the V region
Cytokine for T cell stimulation?
IL2 (it's receptor is CD25)
Which chromosome is C1-INH on?
Molecular mimicry: Coxsackie B virus
Molecular mimicry: Hep B virus
Major Basic Protein
Molecular mimicry: HSV
What are the 5 types of PRR?
LRR, RLR, Ddx, Pyphin, CLR
What does TIR stand for?
Toll IL-1R domain (on LRR)
Which TLR is triggered by influenza?
Which TLR is triggered by flagellin?
TLR5 = flaggelin
Which TLRs are associated with Heat Shock proteins?
TLR 2, 4, 6 (all extracellular)
Which is the TLR that does not use IRAK4?
TLR3 does not use IRAK4/IRAK1/IRAK2
What is the next step after MyD88 stimulation in the pathway?
MyD88 --> IRAK4/2/1 --> TRAF6 --> NEMO --> NFkB
Which TLR is associated with CD14?
TLR 4 is associated with CD14 (there is a #4 in both)
Which NLR is associated with Crohn's disease?
NOD2 is mutated in Crohn's disease
What does STING stand for?
STING: Stimulator of INF Genes protein; abn in SAVI
What does NALP stand for?
Nacht domain leucine-rich repeat and PYD containing proteins
What does RLR stand for?
RNA-sensing RIG-I-like receptors
What does SAVI stand for?
SAVI: STING-associated vasculopathy with onset in infancy
What kind of mutation leads to SAVI?
GOF mutations of STING leads to SAVI
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
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
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
What happens when there is a breakdown CLR anti-candida pattern?
Chronic non-invasive candidate infections
What is the difference bw Dectin 1 and CARD9 mutation?
Dectin 1: strictly mucocutaneous
CARD9: some invasive infection, dermatophytes
What cells are CD3- and CD56 +
Which cytokine do NK cells require for DEVELOPMENT?
NK cells require IL-15, EOMES
Which HLA is prominent in gestational trophoblasts?
HLA-G (babies say gaga)
Which lymphocyte is most prevalent in pregnant decide?
The NK cell
Which chromosome is KIR inherited on?
Which chromosome is MHC inherited on?
What are the transcription factors needed for ALL ILCs?
NIFL3, TOX, Id2 (NIFty TOXic IDeas)
What infections are TLR3 deficiency more prone to?
TLR3 def: HSV encephalitis; influenza cerebrates (CNS inflammation, decrease in IFN-lambda production)
What is the inheritance of TLR3 def?
What is deficient in production in TLR3 def?
INF alpha, beta, lambda
UNC93B def inheritance?
TRIF/TRAF3/TBK1/IRF3 def infections?
What does Dectin1/2 induce in CMCC?
Which diseases present with Oculocutaneous albinism?
Oculocutaenous albinism: Chediak Higashi, Griscelli syndrome, Hermansky Pudlak
How to differentiate Chediak Higashi, Griscelli syndrome, Hermansky Pudlak ?
BLEEDING in Hermansky Pudlok because of platelet granule defect
CNKD1 mutation and inheritance?
GATA2 - AD
CNKD2 mutation and inheritance?
MCM4 (mini chromosome maintenance complex 4)- AR
FNKD1 mutation and inheritance?
FCGR3A (CD16) - AR
What is missing in GATA2 def? CD 56 dim or CD56 bright?
CD56 bright are missing from GATA2 def (CNKCD1)
What kind of killing is affected in FNKC def?
ADCC intact; spontaneous cytotoxicity decried; EBV cells not killed
What are the Src family kinases?
lyn, lck, fyn
What are the Syk family kinases?
What are the Btk family kinases?
What are the inhibitory phosphatases of ITIM?
SHP-1, SHP-2, SHIP
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)
IL-12RB1 mutation leads to which type of infections?
IL-12RB1 def leads to salmonella and atypical mycobacteria
What kind of infections does IRAK4 def lead to ?
IRAK4 def: pyogenic infections: Strep pneumo
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
What type of cell does IL2 promote?
IL2 promotes Treg cells
IL5, what does it promote?
IL5 promotes eosinophils and production of IgA Ab
Which two cytokines promote IgE class switching?
IL4 and IL13
Which cytokines promotes mucus formation?
How does BAFF work?
Upregulates Bcl2 which is an anti-apoptotic protein
How does APRIL work?
Upregulates Bcl2 which is an anti-apoptotic protein
What is the TSLP receptor?
TSLPR and IL7Ra
What is the basophil differentiating cytokine?
IL3 --> basophils
What does CD23 (FceRII) bind to (Ig and CD marker)?
CD23 (FceRII) binds to IgE AND CD23
What does the basophil activation test evaluate?
Basophil activation: evaluates for CD63 or CD203c
What is the tryptase measurement?
>1.2x baseline tryptase + 2 microgram/mL
How to test for NK cell cytotoxicity?
Chromium release assay (Cr51); check for CD107a
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
Deficiency of what part of the complement system leads to recurrent meningococcal infections?
Defects in terminal complement C5-C9 --> meningococcal infections
Which part of the IL2R makes it high affinity?
What IL receptors is gamma chain (gc) a part of?
gc = IL2, IL4, IL7, IL9, IL15, IL21
What two components are part of the IL13R?
IL4Ra and IL13R
What two components are part of the TSLPR?
IL7Ra and TSLPR
IL-18 aka? Which IL is is similar to?
IL18 aka IFN-g inducing factor ; most similar to IL1b
Which adhesion marker does IL18 induce?
What diseases have elevated IL6?
Castleman's dz; Atrial myxoma; MM; RA, JIA
IFNg deficiency results in susceptibility to which types of infections?
M. tb and other intracellular bacteria
What receptor does dupilumab act?
IL4Ra which is on IL4 and IL13
What are the chemokines for lymphocyte homing?
CCL19/CCL21 --> CCR7
What are the chemokines for pro Th2 response?
CCL17/CCL22 --> CCR4
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)
What is the function of L selectin?
L selectin; on Lymphocytes and Leukocytes; GLYCAM - to LN HEV; and MadCAM to PMN rolling
What is the importance of B integrin family a4b7?
Binds to MADCAM - important for gut homing
ICAM1 CD name
ICAM1 = CD54 (think 5=4 = 9, which is almost 10)
ICAM2 CD name
ICAM2 = CD102 (has a 2 in the name)
ICAM3 CD name
ICAM3 = CD 50 (3 and 5 both prime numbers)
ICAM4 CD name
ICAM4 = CD242 (242 has 4 and 4 is a multiple of 2)
What does ICAM1 bind?
LFA1, MAC1, rhinovirus
What does ICAM2 bind?
What does ICAM3 bind?
LFA1 and CD18
What does ICAM4 bind?
LFA1 and CD18
What is the CD name for VCAM1?
VCAM1 = CD106
What does VCAM1 bind?
VCAM1 binds VLA4 - vascular
What does PECAM bind?
CD31 and CD38 - platelets
What is the CD name for PECAM?
PECAM = CD31 (which it also binds, and E looked like backwards 3)
What does MadCam bind?
MadCAM binds a4b7 - mucosa
What does NCAM bind?
Binds VLA4 (a4b1)
What is the CD name for NCAM?
What are the important chemokines in allergy?
CCL2, 5, 7, 11; CXCL8
What proteins inhibit the formation of MAC?
S protein and CD59
Which molecules are considered anaphylotoxins?
C5a, C4a, and C3a
What is C3a chemotactic for?
What is the CD name for CR1?
CR1 aka CD35
What is the CD name for CR2?
CR2 aka CD21 (#2 in both)
What is the other name for CR3?
CR3 aka Mac-1, CD11b/CD18
What is the other name for CR4?
CR4 aka gp 150/95, CD11c/CD18
What is the other name for CD46?
CD46 aka MCP = membrane cofactor protein
What is the other name for CD55?
CD55 = DAF (decay accelerating factor)
What is the other name for CD59?
CD = protectin
What is the ligand for CR1?
C3b, C4b, iC3b (B1)
What is the ligand for CR2?
C3d, iC3b, C3dg (D2)
What is the ligand for CR3?
iC3b and ICAM1
What is the ligand for CR4?
What is the ligand for CD46?
C4b and capsid
What is the ligand for CD55?
C5b-8 and monomeric C9
How is properdin deficiency inherited?
X linked (all other complement def are typically AR)
What is the most common complement d/o seen in SLE?
What is the most common AR agammaglobulinemia?
mu heavy chain
What is a common codominantly inherited blood disorder?
Blood typing (ABO)
ADA SCID T/B/NK?
ADA SCID = (T-/B-/NK-)
Reticular dysgenesis T/B/NK?
Reticular dysgenesis (T-/B-/NK-)
Cernunnos and ligase IV: T/B/NK?
Cernunnos and ligase IV: T-/B-/NK+
Common gamma chain T/B/NK?
Common gamma chain: T-/B+/NK-
CD3 subunit def: T/B/NK?
CD3 subunit def: IL7Ra: T-/B+/NK+
Coronin 1-A def: T/B/NK?
Coronin 1-A def: T-/B+/NK+
CD45 tyrosine phosphatase def: T/B/NK?
CD45 tyrosine phosphatase def: T-/B+/NK+
What are the 2 SCID with increased radiation sensitivity?
Artemis deficiency and Cernunnos/ligase IV: both of which are T-/B-/NK+
What is the abn in reticular dysgenesis?
defect in mitochondrial adenylate kinase2
What are the Es of OmEnn syndrome?
Es: Erythroderma, Eosinophilia, Elevated IgE
Wiscott Aldrich gene mutation?
WASP gene mutation
At what age does alpha fetoprotein increase in Wiskott Aldrich?
Increased alpha fetoprotein after 6-12 months
CD40L deficiency inheritance?
Xlinked - CD40L
CD40 deficiency inheritance?
NEMO deficiency inheritance?
Which def one presents with neutropenia: CD40L, CD40, NEMO
What are the two enzymes in B cells needed for class switching?
AID and UNG
How is NEMO inherited?
NEMO = Xlinked
X linked SH2D1A/SAP gene
X linked defect in XIAP gene
XMEN: X linked immunodeficiency with mag defect
What is RALD and what mutation is seen?
RALD: RAS Associated Leukoproliferative Disorder: NRAS/KRAS mutation; nil DNTs
What is the mutation in APECED?
X linked FOXP3 mutation
How is BTK mutation inherited?
BTK = X linked
How is mu heavy chain inherited?
AR - the most common of AR agammaglobulinemia
What are AD forms of agamm?
E47, AKA, E2A, TCF3
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.
CD27+ is on which type of cells
CD27+ are memory B cells
CD 27 are part of which cytokines family?
CD27 part of the TNF family of cytokines
CD21 aka C3d receptor
What are the ligands for TACI?
Ligands for TACI receptor on B cells are BAFF and APRIL
What is the function of AID?
AID - deaminated cytosine to uracil (C->U); receptor editing function
What is the function of UNG?
Removes Us; function in receptor editing
How are CD21, CD19, CD81 defects inherited?
What class of meds can cause IgA deficiency?
IgA def: anticonvulsants: phenytoin, carbamazepine, valproic acid
Which IgG subclass def is assoc with IgA def?
IgG2 subclass def
When is the IgG nadir in children?
IgG nadir is about 3-6 months (400mg/dL)
What is the inheritance of ELANE mutations?
What results from a Lyst mutation?
Lyst mutation: primary and secondary granules fuse - giant granules (which is Chediak Higashi)
Which mutations cause lack of phagocyte secondary granules and lack of defensins?
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
What is the Rx for CGD?
antifungals, abx, INFg, steroids
Difference in neutrophil function in LAD1 vs LAD2?
LAD1: defect in adhesion, sticking vs LAD2: defect in rolling
What is the cytokine that induces eosinophils?
What is the cytokine that induces mast cells?
What is the cytokine that induces basophils?
What are the transcription factors for basophils?
C-EBPa and GATA-2
What are the contents of a basophil?
histamine, IL4, IL13, TSLP
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)
Can mast cells regranulate?
Yes - mast cells can regranulate
Which mast cell type is deeper in the tissue versus more superficial?
Deeper: MCtc; more superficial: MCt
Which mast cell responds to C5a?
MCtc (Basophils also respond to C5a)
Which mast cell responds to PAF?
Which mast cell responds to opiates?
Which mast cell responds to substance P?
Which mast cell responds to antigen?
MCtc vs MCt: respond to which triggers?
MCtc: responds to more triggers (substance P, Ag, ipiates, C5a); MCt: responds to PAF
What cell responds to f-met-leu-phe?
What are the two cytokines tested in basophil activating test?
CD63 and CD203c
What is CD63?
CD63 on basophils - protein coated on the inside pf secretory granules
What is CD203c?
CD203c on basophils - ectoenzyme, cleaves nucleotides outside the cell
What is CD name for IL3R? (to help ID basophils on basophil activating test)
IL3R = CD123
What cells do basophils not make?
Does omalizumab bind free or bound IgE?
omalizumab binds free IgE
On which cell foes omalizumab reduce IgE receptors on: mast cells of basophils?
What type of tryptase is released with anaphylaxis? *****
B tryptase is released with anaphylaxis
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
True or false: Scabies can cause eosinophilia
True, scabies can cause hypereos
What is the CD marker for selecting?
CD62 P, E, L
In GATA2 def, which CD56 are low, bright or dim?
GATA2 def = low CD56 bright (GATA2 expressed in CD56 bright cells)
Common gamma chain inheritance?
What is the side effect of mepolizumab?
Herpes zoster - consider immunizing beforehand
Which biologic has adverse side effect of candidiasis?
Secukinumab (used for psoriasis, IL17A is the target) - blocks the IL17 pathway leading to candidiasis
Which biologic has a side effect of niesserial infections?
Eculizumab (C5 inhibitor, used for PNH, aHUS), blocks C5 and terminal complement pathway
Which biologic has alpha gal picture?
Cetuximab (EGFR blocker), makes IgE Ab to galactose-a-1, 3-galactose, sensitization to the lone star tick
Zinc deficiency clinical phenotype?
periorificial and aural (limbs) dermatitis, alopecia (loss of hair) and diarrhea
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
What is the MAO of Rapamycin?
Which medications are calcineurin inhibitors?
Tacrolimus, Sirolimus, and Pimecrolimus
Mnemonic for LIVE vaccines
LMNOP RSV; ROME Is MY Best Place To go
RA, Neutropenia, and Splenomegaly (FELTY RANS)
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
What is the genetic defect in Comel-Netherton Syndrome?
SPINK5 - which encodes LEKT1
cathelicidin, antimicrobial peptide that is decreased in eczema; important in antiviral (think eczema herpeticum and vaccinatum)
Which coxsackie strain is related to eczema coxsackium
What is eczema vaccinatum?
disseminated live vaccinia virus from smallpox vaccination (no one gets smallpox anymore in US because it's been eradicated)
Which antimicrobial peptides are NOT unregulated in AD but should be?
LL-37 (cathelicidin) and HBD2-3 (human B-defensins)
Which two cold urticarias have a low CH50?
low CH50: cyroglobulinemia and leukocytoclastic vasculitis
Systemic atypical acquired cold urticaria
Localized or generalized urticaria with cold exposure; negative
Cold dependent dermatographism
Dermographic whaling after stroking pre-cooled skin
Cold-induced cholinergic urticaria
Exercise in cold environments leads to generalized urticaria
Delayed cold urticaria
Urticaria develops 12-48 hours after application of cold provocation
Localized cold reflex urticaria
Positive immediate urticarial response at a site distant to cold provocation
FCAS: Fam cold auto-inflamm sx
Pseudourticaria, fever, arthralgia, conjunctivitis occurring 1-2 hours after cold exposure; AD mutation in NLRP3
PLAID: Phospholipase Cg2 associated def and immune dysregulation
Cold urticaria in childhood, Ab def, autoimmunity. Positive Evaporative cooling test
How to physically test for PLAID?
Positive evaporative cooling test; neg. ice cube test
What is the mutation in PLAID?
mutation in PLCG2
Which type of solar urticaria is assoc with erythropoietic protoporphyria ?
Type 6 VI
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)
Factor I def.
Ar, urticaria, decrease Factor I (C3b inactivator), dec C3 levels
What is SERPING1?
SERPING1 = C1-INH gene in hereditary angioedema
Treatment in Giant cell arteritis?
STEROIDS!! Need to decrease risk for visual loss
What is the treatment for Kawasaki disease?
IVIG x 1 and aspiring: decrease the risk of aneurysms
What diseases can polyarteritis nodes be associated with?
Hep B or Hep C
Which HLA is associating with Abacavir Sn?
Abacavir Sn in white = HLA-B*5701
Which HLA is associating with Carbemazepine Sn in Han Chinese?
Carbepazepine in Han Chinese = HLA-B*1502
Which HLA is associating with Carbemazepine Sn in Europeans?
Carbemazepine Sn in Europeans = HLA-B*3101
Which HLA is associating with allopurinol reactions in Asians?
allopurinol reactions in Asians = HLA-B*5801
Which HLA is associating with insulin, gold, and penicillamine reactions?
Which cytokine is produced by the T-cells in AGEP?
IL8 / CXCL8
Major determinant for PCN allergy
Minor determinant for PCN allergy
Minor: Pcisilloate, penilloate, benzylpenicillin (Pen C)
Which cytokine is related to mucus gland hypertrophy?
IL9 --> mucous gland hypertrophy
Which sinuses drain into the middle meatus?
Anterior ethmoid, frontal, maxillary
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
Where do the posterior ethmoids drain?
Which sinuses do polyps usually arise from?
polyps usually arise from the ETHMOID sinuses
Do anti-leukotriene agents block the early or late phase?
anti-leukotriene block the early and the late phase
Do antihistamines block the early or late phase reaction?
Early and Late phase reaction
What CT finding is seen in empty nose syndrome?
turbinates are very small
Where is a haller cell located?
Haller cell = medial floor of the orbit (basically next to the nose and under the eye.
What is the cell type in Western nasal polyps?
What is the cell type in Asain nasal polyps?
Neutrophilic (elevated IL8)
What is the first line treatment for acute rhinosinusitus?
Augmenting; then doxy or a resp flouroquinolone (moxi)
How long does it take after pet removal for allergen to go back to normal levels?
about 4 months (16-20 weeks)
Which nasal spray is ok to use in pregnancy?
Budesonide; B=baby (cromolyn is also safe in pregnancy)
Which immunosuppressed group can receive the varicella vaccine?
Isolated B cell deficiency
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.
What is the CI for getting Rotavirus vaccine?
CI are: immunodeficiency and h/o intussusception
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)
What is the timing rule for live vaccines?
Either get the live vaccines on the same day, OR get them 4 weeks apart.
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
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
Which vaccines contain yeast?
Hep B (any Hep B containing vaccine)
Which vaccine is contraindicated in egg allergy?
Yellow fever (MMR and influenza are not)
Which vaccines contain Gelatin?
HIZ MYV: HepB, Influenza, Zoster, MMR, Yellow Fever, Varicella
What mutation confers HIV resistance?
CCR5 deletion of bp32 confers resistance to HIV: dbl mutation = resistance, single mutation = slower progression
What is lipocalin also known as?
lipocalin aka calycom
What is a direct bronchoprovocation test?
What is an indirect bronchoprovocation test?
Exercise, cold air, mannitol, adenosine monophosphate
What is considered a positive methacholine challenge test?
< 4 +; 4-16 is borderline
Which cells make IL-12?
IL12 is made by myeloid cells, but not T cells or innate cells
Conventional dendritic cells CD markers
CD1, CD11b/c, CD13, CD14
Plasmacytoid dendritic cells CD markers
Langerhans cells CD markers
Interstitial dendritic cells CD markers
CD11c, CD2, CD9, CD68
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
What is higher in the urine of AERD patients?
LTE4 (because of uncontrolled production of cysLT
Which CD markers doe basophils have?
CD123, CD203c, CD63, CD69
Which cells produce PGD2?
Only mast cells produce PDG2
Platelet Activating Factor: preformed, lipid, or cytokine?
heparin: preformed, lipid, or cytokine?
chondroitin sulfate E: preformed, lipid, or cytokine?
chymotryptase: preformed, lipid, or cytokine?
carboxypeptidase: preformed, lipid, or cytokine?
What is the gene mutation in Familial Cold AutoInflammatory Syndrome?
CIAS1: Cold Induced Auto-Inflammatory Syndrome 1 gene
What is the gene mutation in Muckle Wells Syndrome?
CIAS1: Cold Induced Auto-Inflammatory Syndrome 1 gene
Which cat allergen is related to Pork-Cat syndrome
Fel d 2: albumin
Where does superantigen bind on the TCR?
Do superantigen bind to MHCI or MHCII?
Superantigen bind to MHCII (extracellular bacteria)
What are the Staph superantigens?
SEA, SEB, (staph enterotoxins); TSST (toxic shock syndrome toxin)
What are the strep super antigens?
SPE-A (Strep Pyogenes exotoxins)
Where are cells that express AIRE?
Medulla: Thymic Medullary cells
Where do Tregs develop?
Tregas develop in the thyme medulla
Which TLR binds to mycoplasma?
TLR6/2 - ligand is diacyl lipopeptides
Which TLR binds to synthetic sources?
TLR 7/8 - imidazoquinoloones, synthetic, virus
Which TLR binds to unmethylated CpG motifs of bacteria and viruses?
Serum Sickness is which type of HSN d/o?
Serum sickness if type 3
Which medications do you have to be careful of fluticasone causing Cushing's?
Protease Inhibitors - namely Ritonavir
Which PID is associated with fractures?
What is the major mouse allergen?
Lipocalin: Urine of the male mouse
In which eye disease can you see keratoconus?