Flashcards in IMMUNO Deck (747):
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 aka CCL27 (cutaneous T-lymphocyte attracting chemokine)
- 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-amylase/protein inhibitors
Cupin superfamily (may only give numbers)
7s vicillin and 11s globulins (7/11!)
PR10 protein family
Bet v 1, birch
Atopy leads to increased risk for which agents anayphlayxis?
My EX RADdy was a LATE IDIOt
- Exercise induced anaphylaxis
- Idiopathic anaphylaxis
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); Mycopolyspora - moldy hay, grain, compost
Bagassosis bacteria and what is the agent it's found in?
Thermoactinomyceses sacchari; moldy sugar cane
- Think of a bag and a SACK (sach) of moldy sugar cane
Humidifier fever/AC lung
Thermoactinomyces (T. Vulgarin, T. sacchiri, T candidus); Klebsiella, Acanthamoeba
- KAT loves the humid AC
Hot tub lung
Mycobacterium avian complex (MAC) or Cladosporium - from contaminated water
"Mac clad into the Hot tub"
Basement shower lung
Epicoccum nigrum; mold growing in unventilated showers
Malt worker's lung
- exposure can be malt, tobacco, soy
Cheese worker's lung
Penicillium casei - exposure to moldy cheese
Machine operators lung
Pseudomonas, Acinetobacter, Mycobacter
-PAM operates MY PSEUDO ACting
Bird fancier's lung, pigeon breeder's lung
Avian proteins - exposure to droppings, feathers, serum
Detergent worker's lung
- also seen in egg worker
Alternaria - moldy wood dust
Botrytis cinerea - mold on grapes
Summer type HP
Trichosporium cutaneum - contaminated houses, common in Japan
El niño lung
Pezizia - exposure to mushroom fungus after heavy rainfall
- let's eat pizza during el Nino
- Think Bill Clinton has a yeast infection
Wheat weevil lung/miller's lung
Sitophilus - contaminated flour
- think sito --> silo, wheat in silo
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
Paraphenylenediame is related to which occupation?
MCC cause of contact HSN in hair dressers
Permanent wave solution; one of the MCC contact sensitivity
- does not become less allergenic with washing hair out
What to avoid if sensitive
in topical creams; need to avoid nystatin, aminophylline, and piperazine antihistamines (meclizine and cyclizine)
- does NOT cross react with EDTA: ethylenediamine dihydrochloride
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
ROME Is MY Very Best Place to go AlwayS
Varicella Zoster (chickenpox) and Herpes Zoster(shingles)
Plague (Yersinia Pestis)
Which two sinuses have the most complications in sinusitis?
"My Sweet Friend is complicated"
Which sinuses are most likely to have polyps?
Which allergen is CRS w polyps more likely to be sensitive to?
Kabuki syndrome: mutation and seen in which disease?
A type of CVID - KMT2D mutation
WHIM syndrome mutation
CXCR4 mutation AD - GOF
Where do neutrophil precursors arrest in severe congenital neutropenia
Molecular defect for SCN1
ELANE defect - AD
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; S3CNAB Philly Franc G
- staph aureus
- serratia marcescens
- chromobacter violaceum
- nocardia spp
- aspergillus fumigatus; aspergillus nidulans
- burkholderia cepacia
- Philomegaria Fransicella
- Granulibacter Bethesdensis
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 does 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?
banana, 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=above 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?
5q 31-33 , AD
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 CD markers are mast cells in mastocytosis identified with?
+ CD2 AND/OR +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 scapularis 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)?
C-ANCA/PR3 (proteinase 3)
DONT CONFUSE WITH EGPA
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 type of Ag do NKT and gamma/delta T cells respond to?
The meningococcal and pneumococcal 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 factors 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 (Crypto); 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 3 cytokines do Tregs depend on?
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 thymus medullary epithelial cells
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
What kind of T cells does IL2 stimulate?
Antigen activated T cells; and Treg
Which chromosome is C1-INH on?
Chromosome 11 - SERPING1 gene
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
- also the mutation in Blau's syndrome
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)
- HLA-G and F are present on extravillous trophoblast to protect fetus from maternal immune rejection
Which lymphocyte is most prevalent in pregnant decidua
The NK cell
Which chromosome is KIR inherited on?
Which chromosome is MHC inherited on?
Chromosome 6 (order: Class II, III, I ; 2, 3, 1)
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 cerebritis (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; which cytokine?
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 decreased; 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 and in which function?
IL2 promotes survival and function of Treg cells
IL5, what does it promote (cell and Ig)?
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 basophil differentiating cytokine?
IL3 --> basophils
What is the other name for CD23 and what does it bind to?
CD23 (FceRII) binds to IgE AND B cells
What does the basophil activation test evaluate?
Basophil activation: evaluates for CD63 or CD203c
What is the tryptase measurement calculation?
>/=1.2x baseline tryptase + 2 microgram/mL
- proposed to assess for mast cell activation event
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 inheritance and results in susceptibility to which types of infections?
- AD STAT 1 deficiency
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 to LN?
CCL19/CCL21 --> CCR7
What are the chemokines for pro Th2 response?
CCL17/CCL22 --> CCR4 (seen more on Th2 cells)
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? Which is the most potent for basophil and mast cell degranulation?
C5a> C3a > C4a
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?
CD59 = 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 AKA AK2 (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+
- no thymus regress
CD45 tyrosine phosphatase def: T/B/NK?
CD45 tyrosine phosphatase def: 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 - X-linked
At what age does alpha fetoprotein increase in Ataxia Telangiectasia?
Increased alpha fetoprotein after 6-12 months
CD40L deficiency inheritance?
X-linked - 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
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 including which gene?
AIRE mutation, AR ; 21q22
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 agammaglobulinemia?
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 and what is it's function?
CD21 aka CR2 which is the C3d receptor ; C3b opsonizes Ag --> C3b degraded to C3d --> C3d ligand for CD21, which is part of the BCR complex (CD21-CD19-CD81)
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 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 is the Rx for CGD?
- antifungals: AZOLEs - decreased mortality
- abx: BACTRIM ppx
What is the difference in neutrophil function (rolling, tethering, etc) in LAD1 vs LAD2?
LAD1: defect in adhesion, sticking vs LAD2: defect in rolling
What is the cytokine that induces mast cells?
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 leave 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?
MCtc and MCt
- early phase mediator
Which mast cell responds to antigen?
MCtc vs MCt: respond to which triggers?
- MCtc: responds to more triggers (substance P, Ag, opiates, 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 of secretory granules ; histamine degranulation
What is CD203c?
CD203c on basophils - ectoenzyme, cleaves nucleotides outside the cell; piecemeal degranulation
What is CD name for IL3R? (to help ID basophils on basophil activating test)
IL3R = CD123
What chemical do basophils not make?
Does omalizumab bind free or bound IgE?
omalizumab binds free IgE
On which cell does omalizumab reduce IgE receptors on: mast cells of basophils?
Basophils, mast cells, DC, monocytes
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 OR activating KIT mutation in bx
True or false: Scabies can cause eosinophilia
True, scabies can cause hypereos
What is the CD marker for selectin?
CD62 P, E, L
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, inheritance, and what mineral is deficient?
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
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 abnormally regulated in AD?
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 ice cube test
Cold dependent dermatographism
Dermographic whealing 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 syndrome: Symptoms, inheritance, and mutation
Pseudourticaria, fever, arthralgia, conjunctivitis occurring 1-2 hours after cold exposure; AD mutation in NLRP3/CIAS
PLAID: Phospholipase Cg2 associated def and immune dysregulation: symptoms and test?
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 - AD
Which type of solar urticaria is assoc with erythropoietic protoporphyria ?
Type 6 VI
Which type of urticarias can be 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 aspirin: decrease the risk of aneurysms
What diseases can polyarteritis nodosa 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
Major: BenzylpeniciLOYL (PrePen)
Minor determinant for PCN allergy
Minor: Penicilloate, penilloate, benzylpenicillin (Pen G)
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?
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 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?
Augmentin; 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 antibody product 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.
Which vaccine is the exception to the live vaccine rule (which vaccine may be given)?
Rule is: get same day or 4 weeks apart; but can get Yellow Fever <30 days from MMRV containing vaccines
What if a live vaccine is given < 4 weeks/28 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?
Too Much Running for this Very Yellow Zebra:
Zoster - herpes
What mutation confers HIV resistance?
CCR5 deletion of bp32 confers resistance to HIV: dbl mutation = resistance, single mutation = slower progression
- CXCR4 and CCL3L1 mutations can also have affect on HIV burden
What is lipocalin also known as?
lipocalin aka calycin
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?
< 8 +; 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 does 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, AD: Cold Induced Auto-Inflammatory Syndrome 1 gene
What is the gene mutation in Muckle Wells Syndrome?
CIAS1, AD: 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 (and CD name)?
Vb chain of the TCR (CDR4)
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 do Tregs develop?
Tregs develop in the thymic medulla
Which TLR binds to mycoplasma and what is its ligand?
TLR6 - 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?
Hyper-IgE/Jobs/STAT3 deficiency - AD
What is the major mouse allergen and which part of the mouse does it come from?
Mus m 1: Lipocalin: Urine of the male mouse
In which eye disease can you see keratoconus?
In which eye disease can you see anterior cataracts?
Biopsy in dematographism
dermal edema with a few perivascular mononuclear cells., dilated blood vessels w perivascular cells, normal epidermis, no spongiosis of hyperplasia
How to test HIV infected infant
PCR: at birth, 14-21 days, 1-2 months, and 4-6 months
ELISA: 12 and 18 months (baby still has maternal Ab until 18 mo)
What is the prophylaxis HIV + infants are on from when until when?
PJP prophylaxis from 4-6 weeks to at least 1 year of age
Which of the complement factors ONLY regulates the alternative pathway?
Factor H - is this right?
What is the ligand for MADCAM?
a4b7 for gut homing
What binds VCAM1?
VCAM1 = VLA4 (a4b1)
What is the other name for VLA4?
What does NCAM bind?
NCAM = VLA4
What is the CD marker for dendritic cells?
CD11b/c they all share EXCEPT pDC which have CD123
Which TLR does RSV bind to?
What is the ligand for TLR6?
Which TLR had ssRNA as it's ligand?
Pollutant involved in worse asthma?
Ozone / O3
Pollutant skewing to Th2 ?
Pollutant that is by product of photochemical smog?
What complement tests are low in early complement deficiency (C1q, C1s, C2, C4)?
Which complement tests are low in C3 deficiency?
Decrease CH50, decreased AH50, decreased C3; + C3 Nephritic factor, IgG anti-C3 AutoAb
Which complement tests are low in C5-C9 deficiency?
decreased CH50, decreased specific complement, decreased AH50
Which complement tests are low in Factor B, D, properdin deficiency?
What is the genetic mutation in MBL deficiency?
Which OA agent is associated with welder, chemical/metal workers?
Platinum salts/potassium chromate (think welder holding fancy metal glass with banana juice)
Which OA agent is associated with hairdressers?
Persulfate salts (ammonia persulfate) - think of a hairdresser holding a purse
Which OA agent is associated with adhesives, epoxy resin, paint and plastics?
Which OA agent is associated with plastics/paint?
Which OA agent is associated with shellac, lacquer, plastics, cleaners?
Which OA agent is associated with hospitals and labs?
What phenotype are all the radiosensitive SCID, and name them all?
T-B-NK+ : ARTEMIS, CERNUNNOS, ligase IV, Nijmegen Breakage Syndrome
- Ataxia Telangiectasia is also radiosensitive
HyperIgM1: mutation, inheritance, and germinal centers?
HyperIgM1: CD40L deficiency; X linked; absent germinal centers
HyperIg3: mutation, inheritance, and germinal centers?
HyperIgM3: CD40 deficiency; AR; absent germinal centers
HyperIgM2: mutation, inheritance, and germinal centers?
HyperIgM2: AID; AR; giant germinal centers and lymphoid hyperplasia
HyperIgM4: mutation, inheritance, and germinal centers?
HyperIgM4: UNG; AR; giant germinal centers and lymphoid hyperplasia
Which is the only HyperIgM syndrome that does not have AR inheritance?
HyperIgM1: CD40L, X linked
Ataxia telangiectasia: inheritance and gene mutation?
ATM mutation; AR
Chronic mucocutaneous candidiasis: inheritance and gene mutation?
AIRE mutation; AR
APS-1: inheritance and gene mutation?
APECED: inheritance and gene mutation?
What is TNFS5?
TNFS5 = CD40L
What is the gene defect in DiGeorge Syndrome?
22q11.2 (think CATCH 22, 90%) OR 10p13-14 (renal/GU defect)
What is the autoimmune Ab that may be seen in DiGeorge Syndrome?
How is CTLA4 inherited?
CTLA4 is a haploinsufficiency
Which disease would have low/absent CD15?
LAD2 would have abs/low CD15/Sialyl-Lewis X
Which disease would have low/absent CD18, CD11b?
What gene is abnormal in LAD1?
ITGB2 gene: B2 integrin CD18 - beta chain - , CD11b abnormal
- ITGB2 - "IT Got Bad"
What gene is abnormal in LAD2?
FUCT1, GDP-fucose transporter; CD15/Sialyl Lewis X
What gene is abnormal in LAD3?
FERMT3; Kindlin3 (intracellular beta intern activator)
What is CD79a?
Ig alpha chain on B cells, part of B cell receptor
What is CD79b?
Ig beta chain on B cells, part of B cell receptor
What is CD179?
B cell light chain
Colophony - which industry?
Electronics industry (think of PHONE - electronic)
Which mutation in AAT gives you increased risk of emphysema?
Major Ag in honeybee venom?
Phospholipase A2 (Api m 1)
What is vespula venom standardized by?
Vespula venom is standardized by the amount of hyaluronidase
What does yellow jacket cross reaction with?
White faced hornet, yellow Hornet "Horney yellow jacket"
What drugs decrease the clearance of theophylline?
- also: macrolide Abx and cipro
- decrease clearance = increased level
What does Der p 23 bind to?
Chitin - chitin recognized by TLR2
Which are the ice cube test NEGATIVE urticarias?
- cold induced cholinergic urticaria
- systemic cold urticaria
- cold dependent dermatographism
- FCAS: Familial Cold AutoInfl. Syndrome
(the syndromes, systemic and cholinergic)
What disease can HUVs be associated with?
Which DM and cockroach allergens are tropomyosin?
- Der p 10
- Per a 7
Tropical Derm Purse 107
Major allergen for vespids?
Which antigen can stimulate BOTH T and B cell proliferation?
What is the allergen for celery?
Api g 1 (A pig ate 1)
Which milk allergen is a lipocalin?
Bos d 5: lactoglobulin
What is the allergen Met e 1?
Met e 1 is a shrimp tropomyosin
Which type of allergen involves the peel of the fruit?
lipid transfer protein
Which class of allergen involves the pulp of the fruit
PR-10, Bet v 1
What kind of urticaria is seen in PLAID?
What pairs with cashew?
What pairs with walnut
In AD, does IFNg increase or decrease in the skin?
Which type of mutation is present in AD of filaggrin?
Loss of function mutation: filaggrin
What is the treatment for disseminated eczema herpeticum?
Patients on dapsone should be monitored for which side effects?
What Ab are present in Goodpasture's disease?
Anti-Glomerular Basement Membrane (anti-GBM) Ab
What is the most common side effect of nasal steroids?
local nasal irritation
What is the time duration for chronic rhino-sinusitis?
Greater or equal to 12 weeks (EPOS 2012)
Where do double positive T cells develop?
Where do double negative T cell develop?
Where do single positive T cells develop?
Where do B cells develop before and after birth?
- Before: Fetal Liver
- After: Bone marrow
What is the CD marker for the common gamma chain?
How is the function of IL4, IL5, and IL13 different?
IL4 and IL13 --> class switch to IgE
IL5 --> Eos
Are CD8 T cells involved in acute or chronic graft rejection?
CD8+ CD45RO + cells involved in acute graft rejection
Which cytokine is produced by CD4 cells to help augment CD8 cells?
Encapsulated Bacteria Mnemonic
Some Nasty Killers Have Serious CaBsule ProtEction
- Strep Pneumo / Group B
- H flu
- Salmonella Typhi
- Cryptococcus Neoformans
- Bordatella Pertusis/ Bacilus Anthracis
- Pseudomonas/E coli (some)
Dry/sunny/windy mold Mnemonic
AC2E and BO PITyed SMUT because of his DRESs
- Smut spores
Rainy day mold mnemonic
- fusarium (F looks like R)
Humid Night mold
What does CTLA4 bind to?
CTLA binds to B7-1(CD80)/B7-2 (CD86)
How is TGFb involved in Igs?
TGFb promotes IgA production by inducing B cells to switch to this phenotype
Are B1 cells T cell independent or dependent? What other cells are they analogous to?
B1 cells = T cell 1ndependent , help with polysaccharide vaccines; analogous to delta T cells
Are B2 cells T cell independent or dependent?
B2 cells = T cell dependent , help with protein vaccines
Which chromosome is B2 microglobulin encoded on ?
NK cell recognition; NEK (neck)
Localized to ER and Golgi apparatus (EFG)
Iron metabolism (H-->I)
MHC1 vs MHC2 deficiency: which disease has absent germinal centers
MHC2 deficiency has absent germinal centers
- both have AR inheritance
What is the end result of receptor editing, and is it central or peripheral tolerance?
In B cells, CENTRAL tolerance ONLY in the bone marrow; if too reactive in the bone marrow --> rearrangement and production of new light chain
FasL aka CD98 --> Fas=FasL --> caspase activation
What receptor is downregulated in peripheral B cell tolerance?
CXCR5 is down regulated in B cell peripheral tolerance, inhibiting homing and interaction w B cells
Which are the purine nuceolotides?
Which are the pyrimidine nuceolotides?
What is the earliest B cell to produce Ig?
pre-B lymphocyte earliest to produce Ig
Which of the IgG subclasses is the latest to reach adult levels?
IgG2 is the latest to reach adult levels
Somatic Hypermutation involves which region of Ig?
Class switch recombination involves which region of Ig?
C region; C for class switch
Lck is in which family and binds to what?
Lck in the Src family and binds to CD4 and CD8
Fyn is in which family and binds to what?
Fyn is in the Src family and binds to CD3
What SCID phenotype is Zap70 deficiency?
Which cytokine especially responds to gram negative rods?
TNF especially responds to gram negative rods
Which cytokine does IL-10 inhibit production of?
IL-10 inhibits production of IL-12 (IL-12 promotes TH1 cells)
Which cytokine promotes B lymphocytes into Ab producers?
Which cytokine is required for NK cell differentiation and activation?
Which cytokine induces adhesion molecule ICAM1?
IL-18 induced ICAM1 and other adhesion molecules, leading to enhanced recruitment of leukocytes
Which cytokine is important for resistance of Klebsiella pneumoniae?
IL-23 ; Kleb 23 (Klerb23)
Which cytokine promotes differentiation and maintenance of T cells that produce IL-17?
Which cytokine receptors share common B chain?
IL3, IL5, GM-CSF
Mutation in which gene confers malaria resistance?
GATA in DARC gene confers malaria resistance in African Americans
Which selectins are affected in LAD2 deficiency?
P and E selectin
What is the chemokine abn in Heparin Induced Thrombocytopenia?
What are SIGLECS?
Sialic Acid binding Ig lectin: have ITIM, work to dampen the immune system response (Siglec-8 on Eos)
What are the two types of gravitational samplers?
Durham and Settle (Gravity Settled in Durham NC after the storm)
Which pollen sampler allows you to sample over an entire weekend?
Allergenco Air Sampler: Grab type sampler allows for sampling over an entire weekend
Which pollen sampler orients to the wind?
Burkard sampler, which is a type of gravitational sampler
In Factor H or Factor I deficiency, which complement tests would be low?
low CH50 and low AH50
Which lymph organs is the production of IgE the highest?
tonsils and adenoids
What body secretions is IgA secreted in?
Where are the marginal zone B cells found?
marginal zone of the spleen
Der p 1 size and homology?
Der p 1 : 24 kD and cysteine protease
CXCR5: which cells bind and what is it's ligand?
CXCR5 - expressed on mature naive B cells
CXCR5 binds CXCL13
Function of DNA PK?
DNA-PK = DNA-dependent protein kinase complex: opens up hairpins at coding ends (like Artemis)
Function of ku?
ku: binds broken DNA ends of coding segment and recruits DNA-PK
Endonuclease: removes nucleotides for junctional diversity
DNA ligase function and other name?
DNA ligase aka XRCC4: ligate both coding and RSS ends
Sphingosine - 1 - phosphate receptor (S1PR1)?
CD69: decreases surface expression of S1PR1
Where is CD1 encoded?
CD1 encoded on chromosome 15 (like B2 micro globulin of MHC1)
What is the CRTH2 receptor?
CRTH2 receptor is PGD2 receptor
What are the transcription factors for mast cells vs basophils vs eosinophils?
mast cells: GATA2
Where do eosinophils mature (BM vs Tissue)?
Eosinophils mature in the bone marrow, just like basophils. Mast cells mature in the tissues
Which cells is the trimeric form of FceRI on?
DC, monocytes, and eosinophils
Which allergen cleaves FceRII (CD23)?
Der p 1 cleaves FceRII
Does omalizumab block the early or late phase reaction?
Omalizumab blocks early and late reactions
Where does IgE bind to FceRI?
IgE binds to FceRI by the alpha chain
CD marker for C5a?
C5a = CD88
Basophils are present in early or late allergic disease response?
Basophils = late response
CD 69 and basophil release?
CD69 is NOT associated with degranulation; rather with TLR stimulation (NON-allergen stimulation)
Primary granules of Eosinophil and what is their other name?
Charcot Leyden crystal aka Galectin-10
What are the two main chemokines for neutrophils?
- CXCL8 (IL8)
- LTB4 (B looks like 8; 4x2=8)
Mnemonic for Epithelial cell layers
Before Signing, Get Legal Counsel
- Basale (bottom)
Which cytokines do keratinocytes/epithelial cells secrete?
TSLP, GM-CSF, IL25, IL33 (IL33 part of the IL1 family)
Where are hassall's corpuscles located (be as specific as possible)
Hassall's corpuscles are located in the thymus medullary
What are the immune privileged sites of the body?
BAT: Brain, Anterior Chamber of eye, Testis
Where are the largest number of lymphocytes found?
#2: bone marrow:
#3: GI tract
Which vessels carries lymphocytes into the spleen?
How much mucus is produced in an adult nose per day?
Where does the nasolacrimal duct drain?
CCL27: aka and receptor?
CCL27 aka CTACK; receptor = CCR10
Bullous Pemphigus or pemphigoid has prominent pruritus?
Bullous pemphigoid has prominent pruritus
When does herpes gestationis/pemphigoid gestationis occur and symptoms?
- occurs in 2nd trimester; pruritus and tense bullae
What molecule are defensins abundant in?
Cysteine rich peptides, abundant in neutrophils
Which phase of a clinical trial is submitted for FDA approval?
- after phase 3
In which phase of clinical trial is long term safety tested in?
Phase 4: "post marketing", may find adverse reactions outside the clinical trials
What are the phases of clinical trials and what are the checking for?
3: Confirm finding in large patient population --> FDA approval
4: Long term safety in a diverse population; "post marketing"
Type I and Type II error difference?
- Type I: no true difference: null hypothesis falsely rejected
- Type II error: true difference: null hypothesis falsely accepted
What % of eosinophils on a nasal smear in NARES?
What are the bacteria that are involved in Atrophic Rhinits?
- Klebsiella Ozaenae
- S Aureus
- Proteus Mirabalis
- Coccobacillus faoetidus ozaenae
- Pseudomonas Aeriginosa
- E Coli
"KOSA the CFO of PM wanted PeACE"
When should AIT be started in AFRS?
AIT for fungal and non fungal allergens should be started 5-6 weeks post surgery
Which eye pathologies are Horner-Tranta's dots seen in?
VKC and AKC
Order of eye diseases that Eos seen in conjunctiva (greatest to least)?
"Giant Vernal AtopieS
Cytokines in Acute and Chronic AD
- Acute: IL4, IL13: "4 suits with 13 cards in each deck, including ACes"
- Chronic IL5, IL12, IFNg: " Education is chronic starting at 5 through Grade 12"
What Vitamin deficiencies can be related to rash similar to Eczema?
Vitamin B6, niacin, Biotin (vitamin B7)
Anti-CCP Ab indicate which disease and high/low severity?
Anti-CCP indicate more aggressive disease in RA (so does RF)
What gene in asthma/atopy has cluster of IL4 that may affect severity?
What % of asthmatics can have their asthma triggered by NSAIDs?
What % of asthmatics also have GERD?
Obstruction parameters for asthma
Very severe <35
Vs GOLD criteria for COPD :
- Gold 0: symptoms only
- Gold 1: FEV1 >/= to 80; mild
- Gold 2: 50-79; moderate
- Gold 3: 30-49; severe
- Gold 4: <30 or < 50 with chronic respiratory failure present; very severe
What factors increase FeNO levels ?
Increase FeNO levels:
- Viral URI
- Nitrite Rich foods: hot dog, salami, bacon, baloney
- Spinach, green bean, carrot, beet: (2 green, 1 orange, 1 beet: Think of Barney (purple w green middle) eating carrots, spinach
Foods with sulfites
- think beer/wine/processed foods:
- processed potato
- dried fruit
- pickled peppers
- maraschino cherries
- pickled veggies
"Beer, Wine, Lemon/lime"
Linear epitope for allergens characteristics
Allergen is stable and persistent; more prolonged allergy
Confirmational epitope for allergens characteristics
Allergen is unstable; mild transient allergy
What are the two foods that there are 95% NPV cut off for?
Egg and milk
What are the cut off for EGG for 95% NPV?
- <2 y/o: IgE > 2
- >2 y/o: IgE> 7
What are the cut off for MILK for 95% NPV?
- <1 y/o: IgE > 5
- >1 y/o: IgE> 15
Which peanut component goes along with oral allergy syndrome?
Ara h 8
% of children who outgrow Peanut allergy and ages likely to outgrow
20% by age 6; unlikely to outgrow after age 10
% of children who outgrow tree nut allergy and rule about outgrowing
9%; unlikely to outgrow if allergic to more than 1 TN
LEAP study inclusions and cut offs
- Egg allergy or severe eczema or both
- Serum IgE: <0.35 --> home challenge; >0.35 --> SPT
-- 0-2mm wheal : home intro
-- 3-7 mm wheal: supervised challenge
-- >/= 8mm wheal: allergic
What % of anaphylaxis does biphasic response occur?
- 20% of anaphylactic episodes have biphasic reaction
How to differentiate exercise anaphylaxis and cholinergic urticaria?
Cholinergic urticaria can occur with Passive Warming
How long will serum vs urinary histamine be elevated for?
- serum: 5-60 minutes
- urinary: 24 hours
How long it tryptase elevated for?
- up to 5 hours
What is the mechanism and reaction of oversulfated chondroitin sulfate?
- contaminant in heparin
- activation of contact system by C5a
- rxn: hypotension, AE, no urticaria or pruritus
Latex allergens specific for health care workers?
Hev b 5, 6, 7: "5-6-7 Health!"
What is the key allergen for Dolichovespula arenaria / maculata (hornet)?
Dol m 5 - Antigen 5 (hornet)
What is the key allergen for Vespula?
Antigen 5 - Ves v 5
% of + venom ST with negative serum IgE
% of + venom serum IgE with negative ST
- % of + venom ST with negative serum IgE: 20%
- % of + venom serum IgE with negative ST: 10%
What is the official name for Bed Bugs?
Cimicids, Cimex lectularius
- bite In a row: "breakfast, lunch and dinner, all in a row"
PUPPP: Pruritus Urticarial Papules and Plaques or Pregnancy - what areas does it spare? What is the Rx?
- PUPPP: is erythematous papule within abdominal striae
- SPARES: spares umbilicus, face, palms, soles
- Rx: loratadine, cetirizine
What percentage of new asthma diagnoses in the US are occupational ?
9-15% of new asthma diagnoses in the US are occupational
Which test, if done at work and negative, excludes OA?
Methacholine challenge: should be done when a patient is symptomatic and at work
Organic Dust Toxic Syndrome (ODTS)?
Is ODTS or farmer's lung more common?
- from dust exposure that contains toxin-producing fungi (grain, hay, textiles)
- lack of ST to common fungi
- ODTS is 50X more common than farmers lung
Toxic fume bronchiolitis: what exposure?
Nitrogen dioxide and sulfur dioxide
Silo unloader's disease: what exposure?
- acute exposure of NO2 leading to asphyxia
Byssinosis: what exposure?
- dust inhalation of cotton, flax, hemp
What is Myroxylon pereirae?
Balsam of Peru
What is the spot test for nickel called?
- pink = positive
What flavorings does Balsam of Peru cross react with?
What plant does Balsam of Peru cross react with?
- Cinnamon and vanillin
- pine tree
- may cross react with Colophony (also made from pine trees)
Most common preservative in the US, and is it formaldehyde releaser or not?
Quaternium 15: formadehyde releaser
What is colophony made from?
`Colophony made from pine trees
What are the four classes of steroids?
Antifibrinolytic Side Effects
- postural hypoTN
- myalgia, myositis
- muscle cramps
What SCID phenotype is PNP deficiency?
Which PID has skeletal abn and rachitic rosary?
ADA SCID : T-/B-/NK-
What is PNP deficiency from?
defective purine nucleotide metabolism:
Which radiosensitive SCID has an absence of Hassall's corpuscles on biopsy?
Nijmejen breakage syndrome
What kind of blood products can SCID babies get?
CMV negative, irradiated
- RNF168 mutation
- increased IgM and low IgG: "How funny it is like HyperIgM"
- E Coli and HSV infection
Job's Syndrome/Stat3 deficiency inheritance
What is Tyk2 syndrome and how is it inherited?
- similar to DOCK8 with disseminated BCG lymphadenitis and salmonellosis
How is Wiskott-Aldrich inherited?
What do the germinal centers in XLA look like
XLA germinal centers: small/absent lymphoid tissue, no germinal center
P-delta Activating mutation with Senescent T cells, LAD and Immunodeficiency
- Recurrent infections starting in childhood, chronic EBV, increase in EBV lymphoma
- MTOR is excessively activated
Most familial CVID are inherited in which way?
Familial CVID = AD
What class of drugs can cause IgG subclass deficiency?
Mutations in CGD
- gp91 - CYBB, X-linked, 65%
- gp47 - NCF1, AR, 25%
- gp22 - CYBA, AR, 5%
- gp67 - NCF2, AR, 5%
- gp40, NCF4, AR, <1%
What are the subunits of IL-12?
IL-12: p35, p40
What are th subunits of IL-23?
IL-23: p19, p40
Which cells are CCR5 on ?
Which cells are CXCR4 on?
What can gp120 bind?
gp-120 can bind DC-SIGN
Which chemokine is implicated in EOE?
Eotaxin-3; induced by IL-13
How long should a patient be on PPI before checking biopsy for EoE?
8 weeks/2 months
IgE value for 95% PPV for Peanut?
>/= to 14
IgE value for 95% PPV for Fish?
>/= to 20
Which layer of the GI tract can eosinophils be found normally?
Found in the lamina propria normally, not the surface epi
- Eos NOT normal in the esophagus
Meeting how many of the criteria is needed for diagnosis of mastocytosis?
1 major and 1 minor
Which TLRs are involved in killing mycobacterium?
TLR2 and TLR4
What is the presentation of children with croup?
- barking cough
Which cytokines induce Th17?
IL1B, IL6, IL21, IL23, TGFB --> Th17
Which immunomodulator requires a yearly eye exam?
What is the Ig involved in immunohematologic disease?
(IgM usually involved in auto-immune disease)
How to prevent nonhemolytic febrile reactions?
Removed leukocytes before storage; helps decrease cytokines release of TNFa and IL1b which play a role
How to prevent urticarial transfusion reactions?
Washing future blood products; IgE mediated
How to prevent anaphylactic transfusion reactions?
Twice washing RBCs or use product from IgA deficient donor
How to prevent GVHD transfusion reactions?
Gamma irradiation of cellular blood product
Which receptor for NK cell and ADCC?
FcGRIIIA; binds ONLY to clustered IgG, not monomeric
Which autoantibody is seen in Primary Adrenal failure?
Anti-CYP21A1 (enzyme necessary to make cortisol)
Most common cause of nephrotic syndrome in adults?
Most common cause of nephrotic syndrome in children?
Minimal change disease
Most common glomerulonephritis in the world?
What is the treatment for scleritis?
NSAIDs, steroids, immunosuppression
What does the pupil look like in Anterior Uveitis?
What is the main side effect of colchicine?
GI distress: pain, bleeding; rare: BM suppression
What is the fever syndrome with the longest lasting fevers?
TRAPS (TNF receptor disorder) has fevers for up to several weeks
sarcoid + rash (erythema nodosum) + joints
Does C-ANCA correlate with disease severity in Granulomatosis with Polyangiitis?
Is EGPA related to C-ANCA or P-ANCA?
EGpA = P-ANCA = myeloPeroxidase
Which organ transplants do not require immunosuppression?
cornea, bone, joint tissue
How long after a HSCT can one receive live vaccines?
2 years after HSCT can a patient receive live vaccines
Which SCID does the best after HSCT?
T-/B+/NK- SCID - no conditioning (not including X-linked who have B cells present and non-functional)
Which SCID does the worst after HSCT?
Acute vs Chronic GVHD timeline?
- Acute = first 100 days - CD8
- Chronic > 100 days - CD4
Types of rejection and timing?
- Hyperacute: on the table, minutes to hours; thrombosis
- Accelerated: 2-5 days; thrombosis
- Acute: 7 days - 3 months; CD8
- Chronic: months to years; CD4
Which of the stem cells take the longest to engraft?
Umbilical cord cells take the longest of the stem cells to engraft
Which foods should CF patients avoid?
CF patients should avoid soy because of glutathione dysfunctions
Which HLA , cytokines, and complement regulatory proteins are placental specific?
- HLA-E, F, G
- PGE2, IL-10, Il-4 - regulatory cytokines
- CD46, DAF - prevent complement activation
What time of day is pollen count the highest?
Early morning from 5-10AM
Northern Grass Mnemonic
I grew up in the NORTH, where I got TO Relish Fresh Sweet Blueberries
- Sweet vernal
- Bluegrass (Kentucky)
Southern Grass Mnemonic
Then I moved to the SOUTH for LOVE, where by Boo Bought me Johnsons and a Prarie
Which two allergen types cannot be mixed with pollens?
Mold and cockroach
Which allergen extracts may be able to be placed with high protease activity extracts?
ragweed and cat in 50% glycerin
Effective doses for standardized pollens?
RaCe to the GDP(B)
- RW: 1000-4000 AU
- Cat: 1000-4000 BAU
- Grass: 1000-4000 BAU
- DM: 500-2000 AU
- Bermuda: 300-1500 BAU
- Venom: 100mcg single; 300 mcg mixed
What is the effective dose for dog allergen in AIT?
15 micrograms of Can f 1
"My dog weighs 15 lbs"
What is the effective dose for short ragweed allergen in AIT?
6-12 microgram of Amb a 1
What is the dose of glucagon you would use in a patient who is on B-Blocker and on AIT?
1-5mg glucagon IV push
Dual H1 and mast cell mediators?
Which second gen H1 blockers to use in pregnancy?
- cetirizine, levocetirizine, and loratadine
Which second gen H1 blockers to use in liver disease?
fexofenadine (My liver is Fine)
Which second gen H1 blockers to use in kidney disease?
Which antihistamines give you the side effect of prolonged QT?
FIRST generation antihistamines (chlorpheniramine, diphenhydramine, doxepin, hydroxyzine) have this side effect in high doses
- blockage of potassium current --> prolonged QT --> torsade de points
What are the cardioselective BB?
Which pregnancy category is montelukast?
(Zileuton is category C)
Which anti-leukotriene agent may have liver toxicity?
Which medications do you need to adjust while on Zileuton? ***
Theos War Prop :
- Zileuton inhibitis CYP1A2 - need to decrease the dose of above medications bc Zileuton increases their serum concentration
Muscarinic Receptor functions
M1: eosinophils in COPD; found in ganglia and mast cells
M2: Inhibitory rec on parasympathetic nerves: decreased in viral infections, increased Ach
M2: primary mediator of smooth muscle contraction in human airways - increases intracellular Ca, casting bronchoconstriction
-- H1 blockers can have AntiAch effects via M3 receptor
First line bronchodilator in COPD?
SAMA - ipratropium
Steroid drug metabolism
Cathy TROLls UP the RITunda looking to Kates Birth Control
- increase steroid levels
- Ritonavir (Protease Inhibitor)
Does omalizumab increase or decrease total IgE?
Omalizumab increases total IgE but decreases free IgE
Where does IgE bind on FceRI?
IgE binds at the alpha chain of FcERI
What is the CD marker for CTLA4?
Which disease has Hurthle cells?
Hashimoto's aka Chronic Lymphocytic Thyroiditis
What disease is associated with antibodies against P antigen?
Paroxysmal cold hemoglobinuria
What cells enter the LN through high endothelial venules ?
Naive B and T cells enter through high endothelial venules
Asplenic patient: Prevnar or Menactra first?
Get Prevnar first
Thermoactinomyces sacchari vs vulgaris
- Thermoactinomyces Sacchari: Bagassosis, mold sugar cane
- Thermoactinomyces vulgaris: Farmer's lung
Are CD4 or CD8 cells seen in the parotid biopsy of Sjogren's disease?
How are prions removed from Ig replacement?
Cold ethanol fractionation (modified Cohn-Oncley cold ethanol fractionation)
What are the 3 criteria for aspirin desensitization?
1: FEV1> 70% predicted
2: Montelukast started 1 week prior
3: Patient should continue treatment of ICS +/- LABA Rx
What is the concentration of Epi for SQ/IM and IV administration?
- SQ/IM: 0.01mg/kg of 1:1000
- IV: 0.01mg/kg of 1:10,000
Which vaccines have neomycin?
- Varicella and Herpes Zoster
Japanese Encephalitis Vaccine Reaction
Delayed Urticaria and angiodema
MMR Vaccine Reaction
rash, thrombocytopenia, late fever (5-12 days)
Tetanus Vaccine Reaction
Brachial neuritis, arthus reaction
Pertussis Vaccine Reaction
Febrile seizures, inconsolable crying hypotonic hyporesponsive event
- vaccine contraindicated in patient who had encephalopathy <7 days after pertussis containing vaccine
Yellow Fever Vaccine Reaction
Smallpox Vaccine Reaction
Myopericarditis, eczema vaccinatum
TB skin test vaccine CI
MMR given same day as TB or 4 weeks apart
Steroids and vaccines
<2 weeks on steroids, OK to get
>2 weeks >20 mg/day, wait 1 month
Sulfa antibiotic versus antibiotic: what is the difference?
Non-antibiotic does not have the aromatic amine at the N4 position (and why there is little cross reactivity)
- Sulfa antibiotic: SO2NH2 moiety
Periostin is produced in response to which cytokine?
Reproducibility of FEV1 and FVC?
3 effort that should be within 150ml of each other
What are the SLE Ab?
Which is the most Specific, which is the most Sensitive?
ANA - SNOUT - most sensitive
anti-smith - SPIN - most specific
When can you get Tdap vaccine if pregnant?
Tdap during third trimester
How much bleach per water ratio for eczema?
1/3 cup 8.25% bleach per one tub
Which cell types are CD62L on?
CD62L are on B, T, and NK cells
- on T memory cells, NOT effector cells ***
Which specific CD marker B cells are missing in BTK?
CD19+ B cells
NBT test : positive vs negative:
Positive: changes from yellow --> BLUE (can reduce NBT to formazan)
- negative: yellow - no change
AH50 vs CH50: sheep vs rabbit
- AH50: rAbbit - Alternative
- CH50: sheep jumping over clouds - Classical
Which allergen confers honeybee and vespid cross reactivity?
Which DNA measuring method is used to quantify TRECS?
Which viral infection can decrease the number of TRECs?
HIV1 - TREC numbers improve with HAART
Radiation of XRAY vs CT?
CT has 60-120 times more radiation
Food challenge: what amounts of wet and dry food?
Dry food: 10g
Wet food: 100ml
Double the amount for meat and fish
IgE value for 95% PPV for Soybean?
IgE value for 95% PPV for Wheat?
IgE value for 95% PPV for TreeNuts?
What are the NPV for ST for egg and PN?
= to 3mm
What are the NPV for Serum IgE for egg, milk and PN?
= to 2 (no reaction history to peanut)
- reaction history to peanut =5)
What's the risk of peanut allergy in a child whose sibling is allergic to peanut?
Atopic patient with eczema and food allergy, risk of being allergic to PN?
Risk of being becoming allergic it cut out peanut?
Major grass families
- Northern: Pooiedae
- Southern: Chloridiodae, Panicoideae (Chlorine in the Pain)
How is aspirin made?
Aspirin is made from the bark of a willow tree
Mnemonic for Indoor molds
- Cladosporium (outdoor that moves indoor)
Which allergen cleaves CD25?
Der p 1 and Der p 2
Can DM mix with cockroach or mold?
Yes, DM which has high protease activity, can mix with cockroach or mold (but cockroach and mold cannot be mixed with pollens)
IPEX-like disorder mutation
STAT5b deficiency; low CD25 and low Treg;
What is the receptor for adenovirus?
Lifetime prevalence of eczema for school children?
What immunodeficiency is seen in Ataxia Telangiectasia?
low IgA, low IgE
How do lymphocytes get to GALT?
GALT has no HEV, so lymphocytes get there via afferent lymphatics
What organization is responsible for AIT standardization?
CBER: Center for Biologics Eval and Research