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Hemostasis and Thrombosis
Downstate > HemeOnc > Hemostasis and Thrombosis
93
Medical
Graduate
11/20/2010

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Term
3 ways a platelet can be activated
Definition
mechanical shearing forces, 5-HT, ADP
Term
dense granules contain (2)
Definition
ADP, TXA2
Term
alpha granules contain (1)
Definition
vWF
Term
PF3 is expressed on the __ leaflet upon platelet activation
Definition
outer
Term
in veins, platelets bind ____ in the subendothelium
Definition
platelet integrin receptor
Term
what stimulates expression of gpIIa/IIIb on platelets?
Definition
ADP
Term
what activates factor 7?
Definition
TF
Term
what is the prothrombinase complex?
Definition
factor 10a, cofactor 5a
Term
what activates factor 10?
Definition
factor 7a (extrinsic), factor 9a/cofactor 8a (intrinsic)
Term
what 2 events define tertiary hemostasis?
Definition
1) factor 13 crosslinks fibrin; 2) fibrinolysis begins
Term
what activates factor 12?
Definition
HMWK and PK
Term
what are the factors (in order) of the intrinsic pathway?
Definition
HMWK and PK, 12, 11, 9, 10
Term
what cells produce factor VIII? (2)
Definition
endothelial cells, platelets
Term
what cells produce vWF?
Definition
endothelial cells, platelets
Term
which factors are acute phase reactants?
Definition
vWF, fibrinogen, factor VIII
Term
which factors are vitamin K-dependent?
Definition
2, 7, 9, 10, protein C, protein S
Term
what cofactor is required for gamma-carboxylation of Glu?
Definition
vitamin K
Term
which factor has the shortest half-life and what is it?
Definition
factor 7, 4-6h
Term
which factor has the longest half-life and what is the half life?
Definition
factor 12, 60d
Term
what are the 2 main functions of plasmin?
Definition
1) breaks fibrinogen into degradation products; 2) breaks fibrin into D-dimers
Term
gray platelet syndrome is a deficiency in ___
Definition
alpha granules
Term
delta storage pool syndrome is a deficiency in ___
Definition
dense granules
Term
how does renal failure contribute to thrombocytopenia?
Definition
release of NO --| platelet function
Term
how might the cardiopulmonary bypass machine cause thrombocytopenia?
Definition
causes degranulation of platelets; normal # of platelets
Term
factor 8 deficiency; dx?
Definition
hemophilia A
Term
factor 9 deficiency; dx?
Definition
hemophilia B
Term
factor 11 deficiency, factor levels do not correlate with bleeding; dx?
Definition
hemophilia C
Term
where is vWF stored in subendothelium?
Definition
Weibel-Palade bodies
Term
DDAVP is treatment used for ___
Definition
vWF deficiency type I; it causes degranulation
Term
pt presents with deficiency in extrinsic pathway factor, no excessive bleeding, and slight increase in thrombotic events; dx?
Definition
HMWK and PK deficiency
Term
which vWF deficiency type mimics hemophilia A?
Definition
type 2n
Term
pt presents with anti-factor 8 IgG; what 2 groups of people most commonly present with this acquired autoimmunity?
Definition
multiply transfused and multiply pregnant
Term
what 5 factors increase the risk of a DIC event?
Definition
MOIST = malignancy, obstetrics, infection, snake, trauma
Term
how do PT, aPTT, D-dimer, platelet, and fibrinogen labs look in DIC?
Definition
elevated PT, aPTT, D-dimer; low platelet and fibrinogen
Term
pt presents with PT=15sec and aPTT=40sec; is transfusion indicated?
Definition
No; PT>19s or aPTT>40s
Term
pt presents with PT=20sec and aPTT=60sec; is transfusion indicated?
Definition
yes; PT>19s or aPTT>40s
Term
what is normal PT range?
Definition
10-13s
Term
what are normal fibrinogen levels?
Definition
just kidding. we don't need to know this.
Term
what are normal D-dimer levels?
Definition
please refer to the reference sheet that will be given to us on test day. now go memorize something more important.
Term
pt presents with PT=16sec; which pathway is defective?
Definition
extrinsic pathway
Term
pt presents with PT=14sec and aPTT=39sec; what is the most likely cause of these abnormal results?
Definition
drugs: heparin or warfarin
Term
pt presents with elevated PT and aPTT; mixing study comes back negative (uncorrected); is the clotting abnormality due to a deficiency or an inhibitor?
Definition
inhibitor
Term
factor workup comes back with reduced factor V, VII, VIII, and X; dx?
Definition
DIC
Term
factor workup comes back with reduced factor VII and X, normal V and VII; dx?
Definition
vitamin K deficiency
Term
factor workup comes back with reduced factor V, VII, and X; normal VIII; dx?
Definition
liver disease
Term
what activates protein C?
Definition
thrombin/thrombomodulin complex on endothelial cells
Term
what inhibits factors 5a and 8a?
Definition
protein C/protein S complex
Term
what is the TFPI complex composed of?
Definition
TFPI, TF, 10a, 7a
Term
antithrombin inhibits ___
Definition
factors 2, 10, 9, 11, 12
Term
DVT is characterized by a __-rich, [color] clot
Definition
fibrin-rich, red clot
Term
arterial thrombosis is charactered by a __-rich, [color] clot
Definition
platelet-rich, white clot
Term
unfractionated heparin binds ___
Definition
antithrombin, thrombin, and factor 10a
Term
warfarin inhibits ___
Definition
vitamin K reductase
Term
pt presents with elevated aPTT, history of multiple thromboses, and anti-cardiolipin; dx?
Definition
APLS
Term
pt presents with elevated aPTT, history of multiple abortions, and lupus anticoagulant activity; dx?
Definition
APLS
Term
pt has antibodies against PF4-heparin complex; what do you use for a confirmatory dx? what is the dx?
Definition
5-HT release test; HIT
Term
pt presents with petechiae on the palate, mild bleeding after surgery, bleeding after minor cuts; is this a platelet or a coagulation factor disorder?
Definition
platelet disorder
Term
pt presents with ecchymoses, hemarthrosis, and severe bleeding after surgery; is this a platelet or a coagulation factor disorder?
Definition
coagulation factor disorder
Term
gp1b deficiency; dx?
Definition
Bernard Soulier Syndrome
Term
gpIIb/IIIa deficiency; dx?
Definition
Glanzmann's syndrome
Term
pt presents with thrombocytopenia but normal CBC and smear; dx? tx?
Definition
ITP; splenectomy is most effective, then IV Ig, steroids, anti-RH
Term
pt presents with thrombocytopenia, microangiopathic anemia, schistocytes, normal aPTT and PT; dx? tx?
Definition
TTP-HUS; plasmapharesis
Term
what is the pathogenesis of TTP?
Definition
autoantibodies against ADAMTS-13
Term
thrombocytopenia with thrombophilia (2)
Definition
HIT, DIC
Term
4 T's = ?
Definition
dx for HIT: Timing (5-14d), Thrombocytopenia (>20K), Thrombophilia, not oTher
Term
cryoprecipitate =?
Definition
vWF, fibrinogen, factor 8, factor 13
Term
LMWH binds ?
Definition
antithrombin and factor 10a
Term
unfractionated heparin binds __
Definition
antithrombin, factor 10a and thrombin
Term
tx for vWF deficiency (2)
Definition
DDAVP, cryoprecipitate
Term
factor V Leiden
Definition
factor 5a cannot be deactivated by protein C; pt presents with thrombophilia
Term
hirudin
Definition
thrombin inhibitor derived from leeches
Term
autosomal dominant thrombosis/iron disorders (4)
Definition
vWF, ferroportin, protein C, protein S deficiencies
Term
pt presents with DVT and is put on heparin; 1 week later, her platelet count drops significantly but there is no bleeding; dx? tx?
Definition
HIT; stop heparin, give hirudin for DVT instead
Term
pt bleeds and bruises easily; normal PT and elevated aPTT; history of menorrhagia; dx?
Definition
vWF deficiency
Term
hepcidin: produced by?, expressed by?, function?, upregulated by?
Definition
produced by liver, expressed by enterocytes; increases iron absorption, binds ferroportin to decrease iron export; upregulated by infection, high iron, hemojuvelin
Term
downregulators of hepcidin
Definition
anemia, iron deficiency, EPO, ineffective erythropoiesis
Term
is hepcidin upregulated or downregulated in thalassemia?
Definition
downregulated
Term
ferroportin binds __
Definition
hepcidin
Term
NRAMP1 function
Definition
transports iron out of lysosomes in macrophages
Term
NRAMP2 function
Definition
absorbs iron from gut
Term
hemojuvelin function
Definition
upregulates hepcidin
Term
hephaestin
Definition
oxidizes iron in blood
Term
transferrin function
Definition
carries 2 molecules of iron; binds to TR1
Term
ferritin function
Definition
storage form of iron
Term
when is ferritin upregulated and what is the state of mRNA?
Definition
high iron; IRP does not bind 5' mRNA so transcription can continue
Term
when is transferrin upregulated and what is the state of mRNA?
Definition
low iron; IRP binds 3' mRNA and stabilizes it so transcription can continue
Term
hypochromic, microcytic anemia, high RDW, low ferritin; dx?
Definition
iron deficiency
Term
hypochromic, microcytic anemia, low RDW, high ferritin, low TR1; dx?
Definition
thalassemia
Term
hypochromic, microcytic anemia, high ferritin, normal TR1; dx?
Definition
ACD
Term
Hri is active under [high/low] iron conditions and inhibits globin synthesis
Definition
low
Term
bronze diabetes; dx?
Definition
hemachromatosis
Term
occurs within 6h post transfusion but may resolve 2d later; 10-15% mortality
Definition
TRALI
Term
__ is the most common virus transmitted in transfusions; __ is the most common transfusion-related infection
Definition
CMV; hepB
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