IMMUNO Flashcards
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
Langerhans cells
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
- Radiocontrast
- Latex
- Idiopathic anaphylaxis
Which agent does pre-treatment not help in anaphylaxis risk?
Latex
Which agents does pre-treatment HELP in anaphylaxis risk?
radiocontrast, cold and flueorescien-related anaphylaxis
Farmer’s lung
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
aspergillus species
- 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
Bacillus subtilis
- also seen in egg worker
Woodworker’s lung
Alternaria - moldy wood dust
Winegrower’s lung
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
Saxophonist lung
Candida albicans
- 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
Phthalic Acid
Plastic Worker’s lung
Trimelittic anhydride
Cocamidopropyl betaine
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
Glycerol thioglycolate
Permanent wave solution; one of the MCC contact sensitivity
- does not become less allergenic with washing hair out
Ethylenediamine dihydrochloride
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 Rubella/Rotavirus Oral Polio Measles Endemic Typhus Mumps Yellow Fever Varicella Zoster (chickenpox) and Herpes Zoster(shingles) BCG Plague (Yersinia Pestis) Adenovirus Shingles
Which two sinuses have the most complications in sinusitis?
sphenoid, frontal
“My Sweet Friend is complicated”
Which sinuses are most likely to have polyps?
Ethmoid sinuses
- “PolEEps”
Which allergen is CRS w polyps more likely to be sensitive to?
DM
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
promyelocyte-myelocyte phase
Molecular defect for SCN1
ELANE defect - AD
Molecular defect for SCN2
GFII
Molecular defect for SCN3
HAX1
Molecular defect for SCN4
G6PC3
Molecular defect for SCN5
VPS45
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
- salmonella
- 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
5kpb
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?
Potato
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?
Hiatus Semilunaris
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…
eosinophilic cellulitis
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?
Borrelia burgdorferi
What tick does Borrelia burgdorferi infect?
Ixodes ricinus
Rx for Lyme?
Doxy, cefuroxime, amoxicillin (>8 years old)
Human Monocyte Ehrlichiosis infectious cause?
Ehrlichiosis chaffeensis
Principle vector tick for Ehrlichiosis is?
Amblyomma americanum
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?
Babesia microti
Babesiosis vector?
Ixodes scapularis
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?
Lipid antigens
The meningococcal and pneumococcal vaccines are considered what type of vaccines?
Polysaccharide; conjugated
What are the cytokines of MHC1
IFN alpha, beta, gamma
What are the cytokines of MHC2
IFNgamma
Which components are part of the MHC1 processing pathway?
proteasome, TAP
Calnexin, Calreticulin, Erp57, and tapasin are “chaperone proteins” in which MHC pathway?
MHC1
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?
AR
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?
IL2Ra chain
What 3 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 thymus medullary epithelial cells
BAFF
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?
IgA
Which Ig has the most daily production?
IgA
Best Ig for ADCC?
IgG1
IgG subtype with shortest half life?
IgG3
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
Glutamic decarboxylase
Molecular mimicry: Hep B virus
Major Basic Protein
Molecular mimicry: HSV
Ach receptor
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?
TLR10
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 +
NK cells
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?
Chromosome 19
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?
AD
What is deficient in production in TLR3 def?
INF alpha, beta, lambda
UNC93B def inheritance?
AR
UNC93B infections?
HSV encephalitis
TRIF/TRAF3/TBK1/IRF3 def infections?
HSV encephalitis
What does Dectin1/2 induce in CMCC?
Induces Syk/CARD9
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?
Syk, ZAP-70
What are the Btk family kinases?
Btk, Itk
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?
IL13
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?
IL2Ra
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?
ICAM1
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?
LFA1
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?
CD56
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?
eosinophils ONLY
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?
iC3b
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?
C1q deficiency.
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-)
RAG1/2 T/B/NK?
RAG1/2 T-/B-/NK+
Artemis T/B/NK?
Artemis 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-
IL7Ra: T/B/NK?
IL7Ra: 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?
Autosomal Recessive
NEMO deficiency inheritance?
X-linked
Which def one presents with neutropenia: CD40L, CD40, NEMO
CD40L
What are the two enzymes in B cells needed for class switching?
AID and UNG
XLP1 mutation?
X linked SH2D1A/SAP gene
XLP2 mutation?
X linked defect in XIAP gene
MAGT def?
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
IPEX mutation
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?
AR
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?
C/EBPe mutation
What is the Rx for CGD?
- antifungals: AZOLEs - decreased mortality
- abx: BACTRIM ppx
- INFg
- steroids
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?
SCF
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?
MCt
Which mast cell responds to opiates?
MCtc
Which mast cell responds to substance P?
MCtc and MCt
- early phase mediator
Which mast cell responds to antigen?
MCtc
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?
basophils
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?
Prostaglandins