Friday Heme 4 Flashcards

1
Q

Hepcidin is acute phase reactant synthesized by __
central regulator of __ homeo
stimulated at hig __ levels and inflamm cx
low w hypoxia and inc __

interacts w F__ a protein responsible for transferring IC iron to circulation
w hepcidin, ferroportin is __ dec iron absorption and inhibiting release of iron by __

Regulation of __ absorption is crucial bc it is main mech for iron homeo
iron is absorbed in proximal __ by binding DMT1
then, iron can bind __ and stay in enterocyte, excreted in stool

Iron can bind __ and be released to circ binding transferrin
internalized when binding __ receptors on cells

A

liver, iron
iron
EPO

ferroportin
downregulated
macros

Iron
SI
ferritin

ferroportin
transferrin

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2
Q

recurrent infections w intact T cells and low Ig levels indicates __ as dx
mutation in __ that prevents maturation of b cells

B cells normally aggregate in __ of LN to form follicles
primary are __
secondary are __ w proliferting B cells
in XLA, __ and __ do not form

_ cells are preset in the cortex too
in XLA it will be __ but present
more T cells/dendritic cells present in __ of LN

A

XLA
BTK

cortex
dense
pale
germinal centers, primary follicles

T
small
paracortex

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3
Q

Eukaryotic translation must have binding of __ ribsomal subunit upstream to the __
initiates trsnl at start codon _

in B thalassemia, pt have __/__ anemia
reduce B globin synth causes A chains to __
leads to death of red cell __ and extracellular __
red cells will vary in __/__
may see T/T cells, nucrleated RBC and __ stippling

A

40s, Kozak sequence
AUG

M/H
precipitate
precursors, hemolysis
shape/size
teardrop, target
basophilic
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4
Q

give __ to pt on MTX therapy to stop __ to BM/GI
or on __ therapy to improve cytotoxicity

A__ is cytoprotective free radical scavenger used w alkylating chemo agents
can dec __

DC present antigens to __ cells in the paracortex
becomes enlarged to produce a __ response
will be poorly developed in cx like D__

Medulla of LN contains medullary C_/S_
cord has __/__ cells and M
sinus contains r__ cells and m

A

Leucovorin, toxicity
5FU

amifostine
dry mouth

T
Cell mediated
DiGeorge

cord, sinus

B/plasma, macros
reticular, macros

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5
Q

Nitrates cause poisoning by oxidizing Ferrous iron to __ Fe3+ state
this froms M__, which cannot bind O2
this results in inc affinity of ferrous form for O2, shifting curve to __
pO2 will be __
can see dusky discoloration of __
will see low O2 __/__
delivery/bound fraction are __

Myoglobin is main carrier of O2 in __/__ muscle
similar in structure to heme, but p50 is much __
only has 1 heme group, thus O2 dissociation curve is __

if hemoglobin molecule dissociates, it presents like __

A
Ferric
methemoglobinemia
left
normal
skin
content, carrying capacity
low

skeletal/cardiac
lower
hyperbolic
myoglobin

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6
Q

pt w pancytopenia but normal size __ has aplastic anemia
bone marrow is replaced by __ and stroma
true dx comes w ___

pt w HIV CD4 <200 needs \_\_ for prophylaxis
alternative is \_\_
SE include Fever, R and M
can also induce \_\_\_damage
Bite cells always indicate \_\_ damage
\_\_ bodies are present
A

spleen
fat
bone marrow biopsy

TMP-SMX
dapsone
rash, methemoglobinemia
oxidative
oxidative
heinz
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7
Q

Vinca alkaloids inhibit __ formation, stopping syntehsis of mitotic spindle
replicated chromos cannot __ and segregate, leading to death
__ phase specific agents

Tarry stool and fatigue are due to upper GI bleed from __ agents
cirrhosis/ab pain/jaundice can be seen w M/M
mercaptopurine is __ specific purine analog assc w cholestasis and hepatitis

A

MT
align
M

alkylating agents
MTX, mercaptopurine
S phase

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8
Q

initial attachment of virion __/__ proteins to cell surface proteins is necessary for viral __ and invasion
EBV envelope glycoprotein gp350 allows binding to __ on B cells
this is cellular receptor for __ of complement
if antibody is present, would prevent __ attachment

CMV and other herpesviruses must have initial contact w __ chins on cell surface proteoglycans for cell entry

HIV binds to CD4 proteins via __ glycoprotein
w binding, undergoes coformational change activating __ for fusion

Parvo can bind to human erythroid cells via __ antigen

A
envelope/surface
tropism
CD21
C3d
EBV

GAG
gp120
gp41
P

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9
Q

Pt w recurrent epistaxis, echymoses, marked thrombocytopenia and normal other findings has ___
autoimmune destruction of platelets by __ directed against platelet glycoprotein __
__ process in kids
Secondary may be assc w HIV/__

splenic sequestration occurs w __ spleen

A

ITP
IgG, Gp2B3A
benign
hep C

enlarged

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10
Q

GT is AR disorder of ___
child w __ bleeding
peripheral smear shows no __ clumping
platelet activation releases __ to activate other platelets
Gp2b3a allows for binding to __ and forming a platelet plug
A__ is an inhib, used in unstable angina/ACS/PCI

argatroban is used in H__
Dabigatran is used in A__ and V__

H__ is used in SCD to inc prodxn of HbF
confers protection against __
reserved for __ crisis
Prevent intracellular dehydration of HbS by G__
Ca dependent __ channel, that blocks efflux

A
Gp2b3a
mucocutaneous
platelet
mediators
fibrinogen
Abciximab

HIT
A fib, VTE

hydroxyurea
sickling
pain
Gardos channel blocker
K channel
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11
Q

To dx TTP, must have M and T
all others are __ present

embryonic hemoglobin is composed of __/__ chains
after wk 14, erythpoeisis begins in L/S
HbF is produced, and predominates until __ of life

A2D2 is normal __

A

MHA, thrombocytopenia
infreq

zeta, epsilon
liver, spleen
6m

variant

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12
Q

release of O2 in tissues is enhnced by __ called the Bohr effect
due to h__ side chains
Carbonic anhydrase converts this CO2 to HCO3 and __
H remains in RBC and buffered by __, keeping Hb in deoxygenated state

when deox blood enters lung, rise in O2 inc binding to __
this releases __ from Hb (haldane effect)
HCO3 shif`ts for __ and is converted by CA to CO2 for __ in lung

Higher conc of Cl in __ as compared to arterial

A

CO2
histadine
H
histidine

Hb
H
Cl, excretion

venous

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13
Q

DLBCL on smear shows sheets of __ lymphs w nuclei >5x size of small lymphs

cancer cells, like ab, have resistace to drugs via ATP dependent ___
such as P glycoprotein, from the __ gene
normally found on I/RT cells to eliminate foreign compounds
works well against A

V/D/K may inhibit this protein

A

large

efflux pump
MDR1
intestine, renal tubule
anthracyclines

verapamil, diltiazem, ketoconazole

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14
Q

PV has inc __ mass, inc __/__ and low __
mutation in JAK, which is a __ tyrosine kinase clonal proliferation of myeloid cells

pt w hemolyic anemia, hypercoagulability and pancytopenia indicates __
hemolysis occurs __
CD55/CD59 help to inactivate __ and prevent MAC formation on normal cells

pt gets thrombotic due to release of free __ and prothrombotic factors
may be assc w __ anemia

A

RBC, platelets/leuks, EPO
cytoplasmic

PNH
all day
complement

Hb
aplastic

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15
Q

primary hemochromatosis due to mutation in __ protein
normally interacts w transferrin to act as __ of iron
when inactive, enterocytes sense falsely __ Fe levels
enterocytes inc apical expression of __ to absorb iron
Hepatocytes dec __ synthesis
this inc ferroportin, allowing for Iron to enter __

Integrins are protein receptors that interact w ECM proteins like C/F/L

Fibronectins are large __ made by fibroblast/epi cells
mediates cell __/__

intracellular domains of integrins interact w A/K

A
HFE
sensor
low
DMT1
hepcidin
circulation

collagen, fibronectin, laminin

glycoproteins
adhesion, migration

actin, keratin

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16
Q

VB12 def impairs __ metabolism, w delayed nuclear maturation/division
w replacement, erythrocyte precursors change from M to N
reticulocytes released initially, but begins to __ later

porphyria cutanea tarda caused by ezyme deficiency in __ synthesis
late steps cause photosensitivity w V/B upon sun exposure
due to accumulation of P in skin that reacts w O2
caused by __ def
seen w alcohol/Smoking H/H

Def in ALAD/PBGD do not cause photosensitivity bc problematic metabs do not __
see ab/__ problems

A

Nucleic acid
megaloblastic, normocytic
decline

porphyrin
vesicles, blisters
porphyrinogens
UROD
HIV, HEPc

accumulate
neuropsych

17
Q

deficiency of ALAS dec formtion of __
results in dec __ synth, w M/H red cells
__ is precursor, so def can produce this

Mature B cell leukemia is __
lymphs express CD__ and _

heme synthesis occurs in __ within cells
necessary for 1st and final __ steps
mature RBC have no __ and cannot form Hb
Heme is primarily made in RBC __ in BM or __

A

all porphyrins
heme
VB6

CLL
19/5

mitochondria
3
mito
precursors, liver

18
Q

K___ are proteins necessary for intermediate filaments
found in strat/simple squamous __ and are used as marker

made of large amnts of A/G
coil tightly w lots of __ bonds
also have C__ to form disulfide bonds for rigidity

TK receptors function in the activation of __ pathways for cell growth/differentiation
carcinomas w __ mutation have inc proliferation and resistance to apoptosis
thus, assc w __ prog

A

keratins
epithelium

alanine, glycine, hydrogen
cysteine

transduction
HER2 neu
worse

19
Q

w continued use, tolerance to __ for pain is expected
C/M are SE in which tolerance does not occur
pt on chemo needs prophylactic __ regimen

black pigment stones arise from cx that inc the amount of __ bilirubin in bile
promotes __ bilirubinate precipitation
can be from chronic __ or inc enterohepatic ___

A

Opioids
constipation, miosis
bowel

unconj
hemolysis, recirculation

20
Q

pt w SLE at risk for __ syndrome
labs show __ PTT and false positive __ tests
in vivo, leads to __ state w actvation of phospholipid dependent coag paths
decipher syphillis via __ tests

APS presents w recurrent thromboembolic sx like DVT/PE or __ stroke
unexplained __ loss or __ birth w placental insuff/preeclampsia

should be pos for anti C__ and B_ antibody

A

APS
inc, RPR
hypercoagulable
Treponeum

ischemic
preg, premature

cardiolipin, beta2 glyoprotein 1

21
Q

SCLC can produce __ to cause cushing syndrome
pt has weight _, easy __ and skin __, PUD and __
ACTH stimulates __ receptors to color skin

Pt w wbc > 50000, inc in precursors could have __ or __ rxn
if leuk ALP is low, dx is __
will be normal/inc in __

CML seen w more M__ myelocytic bulge and absolute B/E

A

ACTH
gain, bruising, discoloration, HTN
melanotropin

CML, leukemoid
CML
leukemoid rxn

metamyelocytes
basophilia/eosinophilia

22
Q

breast cancer w __/__/__ mutation is favorable
cancers w __ mutation have a variable course

SCD pt has __/__ hemolysis
bilirubin/lactate are __
haptoglobin is __

pt w SCD also have __ tissue injry/infarcts due to obstruction of small vessels by __ cells
occurs in __, periosteum and deep __
young pt can have D__ of hand foot syndrome, w small infarcts in extremities
see swelling, T__ and warmth, PAIN

young pt bones still have __ bone marrow

A

E/P/BCL2
p53

intra/extra
inc
dec

hypoxic
sickled
bone marrow, muscles

dactylitis
tenderness

hematopoetic