Immunobiolody Flashcards
XLA
due to defects in ___, essential for __ development
typically have low __
absent __ on flow ctyometry
BTK
B cell
Ig levels
B cells
XLA sx present at __, delayed bc of ___
common organisms
Encapsulated bugs __/__
Diarrhea via ___
6m, maternal transfer of IgG
S pneumo, H influenzae
enterovirus meningoencephalitis
Non XLA
__ inherited dz affecting genes requried for __ and __
Tx __ replacement
AR, BCR assembly, signaling
Ig
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
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
Treating CVID
__ replacement
tx ___
limit damage to lung via __
Ig
comorbids
pulm toilet
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
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
T cell/B cell neg
NK+
__/__ is AR
or ___ is AR
Rag1/Rag2
Artemis
T cell neg
B cell pos
NK-
xlinked affects __
JAK 3 is __
IL2RG
AR
DiGeorge syndrome
deletion of ___
defects in development of __ and __
Classic triad
22q11
3/4 pharyngeal pouch
cardiac abnorms
hypoCa (paathyroid hypoplasia)
hypoplastic thymus
Complete digeorge
__ absence of thymus
__ presentation
absent ___
treat w _ transplant
complete
SCID
T cells
thymus
Incomplete digeorge
thymic __
dec __ in early life
do not give __ until normal
inc risk for development to __ later
hypoplasia
T cells
live vaccines
CVID
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
Th0 responds to IL12 producing __ cells
__ activates macros, to kill __ microbes
host defense against ___ and ___ bacteria
__ immunity
Th1
IFNY, intracellular
viruses, intracellular
cell mediated
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
Th0 repsonds to TGFB/IL prodcues __ cells
__ recruits PMN to infection
host defnese against __/__ bacteria
inflam and ___
Th17
IL17
fungi/extracellular
autoimmunity
Th0 responds to TGFB/IL12 produces ___
TGFB blocks activation of __
suppression of __
releases
important in ___
Tregs
lymphs
CD4/CD8
IL10
immunoregulation
HyperIgE syndrome
recurrent ___ abscess
recurrent __/__/__
formation of __ via s aureus and Aspergillus
__/__ candida
severe __
staphylococcal skin
sinusitis/OM/mastoiditis
pneumatoceles
mucosal/nail bed
eczema
HyperIgE presents w ___ in adolescne
retained ___
__
__ joints
fractures w ___
elevated ___
coarse
primary teeth
scoliosis
hyperextensible
minor trauma
IgE
TSS toxin cross links __ and __ leading to augmented ___
B7/CD28, second signal transduction
LAD has defect in ___ to __
leuk adhesion, vessel wall
Defect in phagocytes
development ____
chemotaxis ____
killing __ and ___
severe combined neutropenia
LAD
CGD, chediak higashi
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
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
