Friday Heme 4 Flashcards
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
liver, iron
iron
EPO
ferroportin
downregulated
macros
Iron
SI
ferritin
ferroportin
transferrin
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
XLA
BTK
cortex
dense
pale
germinal centers, primary follicles
T
small
paracortex
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
40s, Kozak sequence
AUG
M/H precipitate precursors, hemolysis shape/size teardrop, target basophilic
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
Leucovorin, toxicity
5FU
amifostine
dry mouth
T
Cell mediated
DiGeorge
cord, sinus
B/plasma, macros
reticular, macros
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 __
Ferric methemoglobinemia left normal skin content, carrying capacity low
skeletal/cardiac
lower
hyperbolic
myoglobin
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
spleen
fat
bone marrow biopsy
TMP-SMX dapsone rash, methemoglobinemia oxidative oxidative heinz
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
MT
align
M
alkylating agents
MTX, mercaptopurine
S phase
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
envelope/surface tropism CD21 C3d EBV
GAG
gp120
gp41
P
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
ITP
IgG, Gp2B3A
benign
hep C
enlarged
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
Gp2b3a mucocutaneous platelet mediators fibrinogen Abciximab
HIT
A fib, VTE
hydroxyurea sickling pain Gardos channel blocker K channel
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 __
MHA, thrombocytopenia
infreq
zeta, epsilon
liver, spleen
6m
variant
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
CO2
histadine
H
histidine
Hb
H
Cl, excretion
venous
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
large
efflux pump
MDR1
intestine, renal tubule
anthracyclines
verapamil, diltiazem, ketoconazole
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
RBC, platelets/leuks, EPO
cytoplasmic
PNH
all day
complement
Hb
aplastic
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
HFE sensor low DMT1 hepcidin circulation
collagen, fibronectin, laminin
glycoproteins
adhesion, migration
actin, keratin
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
Nucleic acid
megaloblastic, normocytic
decline
porphyrin vesicles, blisters porphyrinogens UROD HIV, HEPc
accumulate
neuropsych
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 __
all porphyrins
heme
VB6
CLL
19/5
mitochondria
3
mito
precursors, liver
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
keratins
epithelium
alanine, glycine, hydrogen
cysteine
transduction
HER2 neu
worse
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 ___
Opioids
constipation, miosis
bowel
unconj
hemolysis, recirculation
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
APS
inc, RPR
hypercoagulable
Treponeum
ischemic
preg, premature
cardiolipin, beta2 glyoprotein 1
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
ACTH
gain, bruising, discoloration, HTN
melanotropin
CML, leukemoid
CML
leukemoid rxn
metamyelocytes
basophilia/eosinophilia
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
E/P/BCL2
p53
intra/extra
inc
dec
hypoxic
sickled
bone marrow, muscles
dactylitis
tenderness
hematopoetic
