B&l: 10. Turnicky : Hemostasis Iii/disorders

Front 1

What is the problem in Von Willebrand's Disease?

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Back 1

You do not have vWF, so you cannot bind it to GP on platelets

This prevents a platelet from adhering to non-platelet surfaces

also you will have decreased activity in factor VIII

Front 2

T/F

Symptoms of vW disease are correleated to levels of vWf
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Back 2

FALSE

Symptoms may begin in 2nd decade or at birth with no correlation to levels of vWf

Front 3

what will the PT, TT, and aPTT be like in vW disease?

Back 3

PT, TT, and platelet count are normal

aPTT is prolonged (intrinsic pathway, remember factor VIII)

Front 4

What test will pick up vWF disease?

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Back 4

Platelet function analyzer

Front 5

just to recognize:

Cryoprecipitate and DDAVP are tx for what?

Back 5

Von Willebrand Disease

note: DDAVP: (deamino-D-argenine vasopressin)
induced release of stored vWf in endothelium. (temporary)

Front 6

genetics underlying hemophillia A and B? Factors affected?

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Back 6

X linked

A: VIII
B: IX

note: hemophillia B is also known as Christmas Disease

Front 7

Kid trips and falls, scuffs up knees and they look fine but 24 hours later they come back with a HUGE hemarthrosis..what is going on? Why did they look fine initially but worse later?

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Back 7

Have hemophillia (A or B, would need more info)

primary hemostasis did its job initially and stopped the bleeding but the rest of the cascade never kicked in

Front 8

What is a frontline therapy for someone who has hemophillia (a or b) with life threatening bleeding

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Back 8

Recombinant Factor VIIa-->Novoseven

Front 9

inappropriate formation of fibrin in microcirculation, fibrinogen and other procoagulants (platelets, etc) are consumed faster than it can be produced and there is subsequent bleeding...this describes?

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Back 9

Disseminated intravascular coagulation (DIC)

Front 10

What is a serious problem strongly linked to DIC?

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Back 10

Neoplasms (AML-M3, carcinomas ESPECIALLY LEUKEMIA)

Front 11

Acute DIC leads to=

Chronic DIC =

Back 11

Acute- hemorrhagic (high mortality, bleeding from multiple sites)

Chronic- thrombotic (associated with malignancy)

Front 12

What is the best monitor of DIC?

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Back 12

Decreased platelets

Front 13

what will your D-Dimer be like in DIC?

Back 13

Will always see positive D dimer because you have already kicked off the coag cascade

Front 14

why would you see schistocytes in DIC?

Back 14

Fibrin is being formed in microvasculature, the RBC are coming down and getting sheared off--> schistocytes

Front 15

What is the therapy for CHRONIC DIC?

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Back 15

Heparin if thrombosis, but excess bleeding may occur

remember in chronic you have thrombosis

Front 16

When children are born, they are given a Vitamin K shot. Why?

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Back 16

livers are still immature, provides post-translational modification of II, VII, IX, X, protein C & S in 24hr with administration of Vitamin K

Front 17

What is most greatly affected by vitamin K deficiency?

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Back 17

factor VII (shortest half life)

note: vitamin K is needed for post-translational modification of II, VII, IX, X, protein C & S

Front 18

What can lead to a high degree of placental thrombosis and lead to abortion?

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Back 18

Lupus like Anti-coagulant

seen in SLE

Front 19

Where does Warfarin/Coumadin have its effect? What does this do?

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Back 19

Inhibits the effects of vitamin K dependent factors (II, VII, IX, X, protein C & S)

thus will stop clotting

Front 20

PT test screens for what? good to determine the therapeutic effectiveness of what drug?

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Back 20

Extrinsic and Common

Coumadin

Front 21

Can you compare PT values btw states? If not, What can you use

Back 21

NO

International normalized ratio (INR)

Front 22

Antidote to warfarin?

Back 22

Vitamin K

Front 23

aPTT can be used to monitor what drug?

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Back 23

Heparin

Front 24

Genetically acquired trait resulting in protein C resistance =

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Back 24

Factor V Leiden

leads to hypercoaguable state

note: The function of Protein C is to inactivate Va & VIIIa
FV Leiden not inactivated leading to increased Va in the blood (propensity to clot)

Front 25

Tx for Factor V Leiden?

Back 25

Life Long Anticoag

Front 26

Hughs Syndrome/ Antiphospholipid Antibody Syndromes is associated with what?

Back 26

Associated with both arterial & venous clots-->thrombosis, misscarrage and preeclampsia

hypercoaguable

Front 27

thrombosis, misscarrage and preeclampsia can be associatged with what hypercoagable problem?

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Back 27

Antiphospholipid Antibody Syndromes

Front 28

why is homocystine problematic?

Back 28

Homocysteine is associated with clot formation