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Multisystem 1 > Immunobiolody > Flashcards

Immunobiolody Flashcards

1
Q

XLA
due to defects in ___, essential for __ development

typically have low __

absent __ on flow ctyometry

A

BTK
B cell

Ig levels

B cells

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2
Q

XLA sx present at __, delayed bc of ___

common organisms
Encapsulated bugs __/__

Diarrhea via ___

A

6m, maternal transfer of IgG

S pneumo, H influenzae

enterovirus meningoencephalitis

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3
Q

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

Tx __ replacement

A

AR, BCR assembly, signaling

Ig

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4
Q

CVID
decreased __ and either __ or __

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

Recurrent sinopulm infections w __ and __

never occurs before ___

A

IgG, IgA/IgM

vaccines, polysaccharide

memory CD27

H influenza, S pneumo

2 yo

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5
Q

CVID affects __ growth/acivation

and __/__ interactions

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

mortality via __ such as __
or ___

A

B cell

B cell/T cell

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

malignancy, NHL
lung dz

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6
Q

Treating CVID

__ replacement
tx ___

limit damage to lung via __

A

Ig
comorbids

pulm toilet

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7
Q

B1 B cells produced in __ and __ cavities

aspelic pt needs ___

__ vaccines 14 days prior with ___

need other vaccines such as __ and ___ and __

A

peritoenal/pleural

ab prophylaxis

Polysaccharide, PPSV23

PCV13, MenB, HiB

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8
Q

T cell neg
B cell neg
NK cell neg

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

A

Adenosine deaminase, AR

Purine Nucleoside Phosphorylase, AR

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9
Q

T cell/B cell neg
NK+

__/__ is AR

or ___ is AR

A

Rag1/Rag2

Artemis

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10
Q

T cell neg
B cell pos
NK-

xlinked affects __
JAK 3 is __

A

IL2RG

AR

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11
Q

DiGeorge syndrome

deletion of ___
defects in development of __ and __

Classic triad

A

22q11
3/4 pharyngeal pouch

cardiac abnorms
hypoCa (paathyroid hypoplasia)
hypoplastic thymus

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12
Q

Complete digeorge

__ absence of thymus
__ presentation
absent ___

treat w _ transplant

A

complete
SCID
T cells

thymus

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13
Q

Incomplete digeorge

thymic __
dec __ in early life

do not give __ until normal

inc risk for development to __ later

A

hypoplasia
T cells

live vaccines

CVID

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14
Q

CD3 is marker for ___

CD4 for ___

CD8 for ___

CD14 for __/__

CD19/21 for ___

Cd16/CD56 for ____

A

all T cells

hlper T cells

Cytotoxic T cellls

monos/macros

b cells

nk cells

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15
Q

Th0 responds to IL12 producing __ cells

__ activates macros, to kill __ microbes

host defense against ___ and ___ bacteria

__ immunity

A

Th1

IFNY, intracellular

viruses, intracellular

cell mediated

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16
Q

Th0 Responds to IL2/4 produces ___ cells

__ stims b cell differentiation and __ prodxn

__ recruits eosinophils

host defense against __

__ immunity

A

Th2

IL4, IgE

IL5

parasites

humoral

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17
Q

Th0 repsonds to TGFB/IL prodcues __ cells

__ recruits PMN to infection

host defnese against __/__ bacteria

inflam and ___

A

Th17

IL17

fungi/extracellular

autoimmunity

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18
Q

Th0 responds to TGFB/IL12 produces ___

TGFB blocks activation of __

suppression of __

releases

important in ___

A

Tregs

lymphs

CD4/CD8

IL10

immunoregulation

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19
Q

HyperIgE syndrome

recurrent ___ abscess

recurrent __/__/__

formation of __ via s aureus and Aspergillus

__/__ candida

severe __

A

staphylococcal skin

sinusitis/OM/mastoiditis

pneumatoceles

mucosal/nail bed

eczema

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20
Q

HyperIgE presents w ___ in adolescne

retained ___
__
__ joints

fractures w ___
elevated ___

A

coarse

primary teeth
scoliosis
hyperextensible

minor trauma
IgE

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21
Q

TSS toxin cross links __ and __ leading to augmented ___

A

B7/CD28, second signal transduction

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22
Q

LAD has defect in ___ to __

A

leuk adhesion, vessel wall

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23
Q

Defect in phagocytes

development ____

chemotaxis ____

killing __ and ___

A

severe combined neutropenia

LAD

CGD, chediak higashi

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24
Q

SCN

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

by 2, most have __/__

markedly dec ___

A

early infancy, resp/skin
S aureus, Strep

oral ulcerations/gingivitis

neutrophils

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25
Q

SCN
tx w ____

risk of __ later

A

GCSF

hematologic malignacy

26
Q

LAD
recurrent skin infections w out ___

Omphalitis- delayed separation of ___

develop __/__ over time

markedly elevated __ on CBC

A

no pus

umbilical cord

severe gingivitis/periodontitis

WBC

27
Q

LAD defects in leuk ___

type 1 def in ___ (B2 integrins)

Type 2 abnormality in ___
defetive ligan for ___

type 3 with ___

inherited ___

A

CD18

fructose transporter
selectins

kindlin 3

AR

28
Q

Chediak Higashi syndrome

recurrent __/__ infect in infancy

develop __/__ common

partial ___

__ delay

A

skin/PNA

gingivitis/periodontitis

oculocutaneous albinism

developmental

29
Q

Chediak higashi defect in __ gene

important for trafficking -__ to lysosomes

dx w ___

_ tx heme manifestations

A

LYST

proteins

peripheral smear

stem cell transplatn

30
Q

CGD develops granuloma __
tx w __/__

catalase + orgs
S
S == gram - rod
B = gram - rod

inherited __

A

everywherere
ab/CS

staph aureus
serratia marscescans
burkholderia cepacia

X linked

31
Q

high alcohol intake can lead to __ via __

also __ plays a role

A

leukopenia, bone marrow suppression

folate def

32
Q

classical complement important for clearing __

leads to deposition of __ on complex

binds to __ on RBC

complex stripped of RBC by __ in spleen

A

immune complexes

C3B

CR1

macros

33
Q

Classic complement def
C1
C2
C4 all develop __ dz

slight inc risk of __

A

SLE

infection w encapsulated bacteria

34
Q

C3 def important for __ of response

presents after ___

Freq infections w __
S/H/N

assc w ___

A

amplification of complement

birth

encapsulated orgs
Strep pneumo/Hib/N men

immune complex mediated glomerulonephritis

35
Q

Terminal complement def in __

recurrent __ infections

__ is xlinked for

A

C5-C9

N men/gon

properdin

36
Q

Histamine angioedmea is __ mediated

I
progresses ___
U
resolves w/in __

improves w __/__/__

A

mast cell

itchy
rapidly
urticaria
24hrs

epi/CS/antihistamines

37
Q

Bradykinin angioedema is

P
progresses over __
no __
resolves in __

__ to tx

seen w __/__/___

A

painful
hrs
urticaria
5 days

resistant

aceI/hereditary angioedema/Acquired angioedema

38
Q

Rheumatic fever caused by ___ for pathogen cross reacting w ___

A

ab, self proteins

39
Q

arthus rxn

deposition of __ complexes follwing ___ of substance

leads to local ___

A

ag-ab, intradermal ingestion

vasculitis

40
Q

serum sickness

__ w after exposure
__ with reexposure

F
R
U mediated by ___ (anaphylatoxins __/__)
A/A

A

1-3 w
12-36hrs

fever
rash
urticaria, complement, C3a/C5a
arthralgia/arthritis

41
Q

type 1 rxn antigen is ___

immune reactant is

effector mechanism via

A

soluble

IgG

mast cell

42
Q

Type 2 rxn immune reactant __

cell/matrix assc antigen effector is __ and __ cells

cell surface receptor antien effector mechaism via __ that alters signaling

A

IgG

complement, FcR

ab

43
Q

Type 3 immune reactant __

antigen is ___

effector mech is __/__

A

IgG

soluble

Complement/phago

44
Q

Type 4

Th1 cell- antigen is __
__ activation

Th2 cell- antigen is __
produces __/__/__

CTL- antigen is ___
produces ___

A

soluble
macros

soluble
IgE/eosin/mastocytosis

cell assc
cytotoxicity

45
Q

young male, back pain, SI joint changes, symmetrical arthritis

dx is

assc w

A

akylosing spondylitits

HLAB27

46
Q

Sausage digit, nail changes, psoriasis

dx is

assc w

A

psoriatic arthritis

hlab27

47
Q

IBD/Reactive arthritis assc

A

HLA B27

48
Q

HLA DR3 assc w

A

T1DM
SLE
Graves/Hashimoto/Addison

49
Q

HLA DQ2/8 assc w

A

Celiac dz

50
Q

HLA Dr4 assc w

A

T1DM/RA

51
Q

Hemoptysis + renal dz dx

__ deposits of IgG

__ positive against __

type ___ hypersensitivity

A

goodpastures

linear

anti-GBM, t4 collagen

2

52
Q

Nasal sinus involvement/pul dz, glomerulonephritis dz is

__ positive against

A

GPA

cANCA, PR3

53
Q

asthma, eosinophilia, necrotizing vasculitis w eosinophils is ___

__ involvmet is rare

__ positive against ___

A

Eosinophilic granulomatosis w polyangitis

renal

pANCA, MPO

54
Q 
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

A

3
3, pleural/pericardial
2
3

ANA
Anti DS DNA
Anti Smith

55
Q

drug induced lupus via __ and ___

__ antibodies

A

hydralazine
procainamide

anti-histone

56
Q

symmetric arthritis w morning stiffness, small joints of hand/feet

bony prominences is

early, specific marker

Rheumatoid facotr is ___ directed ab
most commonly ___

A

RA

anti CCP or anti ACPA

anti IgG Fc
IgM

57
Q

Raynauds
Thin fingers
esophageal dysmotility
cutaneous calcium

__ sundrome
__ antibody

A

CREST

anti centromere

58
Q

scleroderma antibody

A

anti SCL 70

59
Q

Polymyositis/dermatomyositis

__ weakness
elevated __

__ positive
anti - ___/__

A

proximal muscle
muscle enzymes

ANA

SSA/Jo

60
Q

MA woman, acute onset hepatitis, high transaminases, ANA positive

dz is ___

positive for __ and __

A

AI hepatitis

anti smooth muscle/actin antibody
anti-LKM

61
Q

MA woman w liver dz, elevated alk phos is ____

___ antibodies

A

PBC

anti mitochondrial antibodies

Multisystem 1 (21 decks)

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