Immunobiolody Flashcards by Nathan Hansen

XLA
due to defects in ___, essential for __ development

typically have low __

absent __ on flow ctyometry

BTK
B cell

Ig levels

B cells

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XLA sx present at __, delayed bc of ___

common organisms
Encapsulated bugs __/__

Diarrhea via ___

6m, maternal transfer of IgG

S pneumo, H influenzae

enterovirus meningoencephalitis

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Non XLA
__ inherited dz affecting genes requried for __ and __

Tx __ replacement

AR, BCR assembly, signaling

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CVID
decreased __ and either __ or __

dec resonse to __ for __ antigens
often dec pops of __ B cells

Recurrent sinopulm infections w __ and __

never occurs before ___

IgG, IgA/IgM

vaccines, polysaccharide

memory CD27

H influenza, S pneumo

2 yo

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CVID affects __ growth/acivation

and __/__ interactions

some get \_\_ such as 
Autoimmune \_\_
Immune \_\_
Rheumatoid\_\_
autoimmune \_\_/\_\_/\_\_

mortality via __ such as __
or ___

B cell

B cell/T cell

AI cx
hemolytic anemia
thrombocytopenia
arthritis
thyroiditis/enteropathy/lung dz

malignancy, NHL
lung dz

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Treating CVID

__ replacement
tx ___

limit damage to lung via __

Ig
comorbids

pulm toilet

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B1 B cells produced in __ and __ cavities

aspelic pt needs ___

__ vaccines 14 days prior with ___

need other vaccines such as __ and ___ and __

peritoenal/pleural

ab prophylaxis

Polysaccharide, PPSV23

PCV13, MenB, HiB

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T cell neg
B cell neg
NK cell neg

leads to ___ def, which is __
or PNP which is ____

Adenosine deaminase, AR

Purine Nucleoside Phosphorylase, AR

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T cell/B cell neg
NK+

__/__ is AR

or ___ is AR

Rag1/Rag2

Artemis

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T cell neg
B cell pos
NK-

xlinked affects __
JAK 3 is __

IL2RG

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DiGeorge syndrome

deletion of ___
defects in development of __ and __

Classic triad

22q11
3/4 pharyngeal pouch

cardiac abnorms
hypoCa (paathyroid hypoplasia)
hypoplastic thymus

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Complete digeorge

__ absence of thymus
__ presentation
absent ___

treat w _ transplant

complete
SCID
T cells

thymus

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Incomplete digeorge

thymic __
dec __ in early life

do not give __ until normal

inc risk for development to __ later

hypoplasia
T cells

live vaccines

CVID

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CD3 is marker for ___

CD4 for ___

CD8 for ___

CD14 for __/__

CD19/21 for ___

Cd16/CD56 for ____

all T cells

hlper T cells

Cytotoxic T cellls

monos/macros

b cells

nk cells

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Th0 responds to IL12 producing __ cells

__ activates macros, to kill __ microbes

host defense against ___ and ___ bacteria

__ immunity

Th1

IFNY, intracellular

viruses, intracellular

cell mediated

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Th0 Responds to IL2/4 produces ___ cells

__ stims b cell differentiation and __ prodxn

__ recruits eosinophils

host defense against __

__ immunity

Th2

IL4, IgE

IL5

parasites

humoral

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Th0 repsonds to TGFB/IL prodcues __ cells

__ recruits PMN to infection

host defnese against __/__ bacteria

inflam and ___

Th17

IL17

fungi/extracellular

autoimmunity

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Th0 responds to TGFB/IL12 produces ___

TGFB blocks activation of __

suppression of __

releases

important in ___

Tregs

lymphs

CD4/CD8

IL10

immunoregulation

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HyperIgE syndrome

recurrent ___ abscess

recurrent __/__/__

formation of __ via s aureus and Aspergillus

__/__ candida

severe __

staphylococcal skin

sinusitis/OM/mastoiditis

pneumatoceles

mucosal/nail bed

eczema

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HyperIgE presents w ___ in adolescne

retained ___
__
__ joints

fractures w ___
elevated ___

coarse

primary teeth
scoliosis
hyperextensible

minor trauma
IgE

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TSS toxin cross links __ and __ leading to augmented ___

B7/CD28, second signal transduction

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LAD has defect in ___ to __

leuk adhesion, vessel wall

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Defect in phagocytes

development ____

chemotaxis ____

killing __ and ___

severe combined neutropenia

LAD

CGD, chediak higashi

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SCN

presents in _ w recurrent __/__ infections
bugs like __ and __

by 2, most have __/__

markedly dec ___

early infancy, resp/skin
S aureus, Strep

oral ulcerations/gingivitis

neutrophils

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SCN tx w ____ risk of __ later

GCSF hematologic malignacy

LAD recurrent skin infections w out ___ Omphalitis- delayed separation of ___ develop __/__ over time markedly elevated __ on CBC

no pus umbilical cord severe gingivitis/periodontitis WBC

LAD defects in leuk ___ type 1 def in ___ (B2 integrins) Type 2 abnormality in ___ defetive ligan for ___ type 3 with ___ inherited ___

CD18 fructose transporter selectins kindlin 3 AR

Chediak Higashi syndrome recurrent __/__ infect in infancy develop __/__ common partial ___ __ delay

skin/PNA gingivitis/periodontitis oculocutaneous albinism developmental

Chediak higashi defect in __ gene important for trafficking -__ to lysosomes dx w ___ _ tx heme manifestations

LYST proteins peripheral smear stem cell transplatn

CGD develops granuloma __ tx w __/__ catalase + orgs S S == gram - rod B = gram - rod inherited __

everywherere ab/CS staph aureus serratia marscescans burkholderia cepacia X linked

high alcohol intake can lead to __ via __ also __ plays a role

leukopenia, bone marrow suppression folate def

classical complement important for clearing __ leads to deposition of __ on complex binds to __ on RBC complex stripped of RBC by __ in spleen

immune complexes C3B CR1 macros

Classic complement def C1 C2 C4 all develop __ dz slight inc risk of __

SLE infection w encapsulated bacteria

C3 def important for __ of response presents after ___ Freq infections w __ S/H/N assc w ___

amplification of complement birth encapsulated orgs Strep pneumo/Hib/N men immune complex mediated glomerulonephritis

Terminal complement def in __ recurrent __ infections __ is xlinked for

C5-C9 N men/gon properdin

Histamine angioedmea is __ mediated I progresses ___ U resolves w/in __ improves w __/__/__

mast cell itchy rapidly urticaria 24hrs epi/CS/antihistamines

Bradykinin angioedema is P progresses over __ no __ resolves in __ __ to tx seen w __/__/___

painful hrs urticaria 5 days resistant aceI/hereditary angioedema/Acquired angioedema

Rheumatic fever caused by ___ for pathogen cross reacting w ___

ab, self proteins

arthus rxn deposition of __ complexes follwing ___ of substance leads to local ___

ag-ab, intradermal ingestion vasculitis

serum sickness __ w after exposure __ with reexposure F R U mediated by ___ (anaphylatoxins __/__) A/A

1-3 w 12-36hrs fever rash urticaria, complement, C3a/C5a arthralgia/arthritis

type 1 rxn antigen is ___ immune reactant is effector mechanism via

soluble IgG mast cell

Type 2 rxn immune reactant __ cell/matrix assc antigen effector is __ and __ cells cell surface receptor antien effector mechaism via __ that alters signaling

IgG complement, FcR ab

Type 3 immune reactant __ antigen is ___ effector mech is __/__

IgG soluble Complement/phago

Type 4 Th1 cell- antigen is __ __ activation Th2 cell- antigen is __ produces __/__/__ CTL- antigen is ___ produces ___

soluble macros soluble IgE/eosin/mastocytosis cell assc cytotoxicity

young male, back pain, SI joint changes, symmetrical arthritis dx is assc w

akylosing spondylitits HLAB27

Sausage digit, nail changes, psoriasis dx is assc w

psoriatic arthritis hlab27

IBD/Reactive arthritis assc

HLA B27

HLA DR3 assc w

T1DM SLE Graves/Hashimoto/Addison

HLA DQ2/8 assc w

Celiac dz

HLA Dr4 assc w

T1DM/RA

Hemoptysis + renal dz dx __ deposits of IgG __ positive against __ type ___ hypersensitivity

goodpastures linear anti-GBM, t4 collagen 2

Nasal sinus involvement/pul dz, glomerulonephritis dz is __ positive against

GPA cANCA, PR3

asthma, eosinophilia, necrotizing vasculitis w eosinophils is ___ __ involvmet is rare __ positive against ___

Eosinophilic granulomatosis w polyangitis renal pANCA, MPO

``` Repro age woman, fever Joint pain type __ HS serositis type ___ HS leading to __/__ Cytopenia type __ HS Kidney dz type __ HS ``` __ is sensitive ___ inc risk of nephritis ___ high specificity

3 3, pleural/pericardial 2 3 ANA Anti DS DNA Anti Smith

drug induced lupus via __ and ___ __ antibodies

hydralazine procainamide anti-histone

symmetric arthritis w morning stiffness, small joints of hand/feet bony prominences is early, specific marker Rheumatoid facotr is ___ directed ab most commonly ___

RA anti CCP or anti ACPA anti IgG Fc IgM

Raynauds Thin fingers esophageal dysmotility cutaneous calcium __ sundrome __ antibody

CREST anti centromere

scleroderma antibody

anti SCL 70

Polymyositis/dermatomyositis __ weakness elevated __ __ positive anti - ___/__

proximal muscle muscle enzymes ANA SSA/Jo

MA woman, acute onset hepatitis, high transaminases, ANA positive dz is ___ positive for __ and __

AI hepatitis anti smooth muscle/actin antibody anti-LKM

MA woman w liver dz, elevated alk phos is ____ ___ antibodies

PBC anti mitochondrial antibodies