ASH review series Flashcards

1
Q

Perioperative management of congenital Factor VII deficient patient

A

Recombinant Factor VIIa

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2
Q

Acquired hemophilia mixing study result

A

No correction. (Not a congenital factor deficiency, acquired antibody)

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3
Q

Factor XII deficiency presentation

A

Don’t bleed

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4
Q

Differential for isolated prolonged PT-INR

A

Mild liver disease →
Congenital factor VII deficiency or acquired factor VII inhibitor → otherwise healthy person +
mild vitamin k deficiency →
Anticoagulant (VKA, direct thrombin inhibitor, ) →
polycythemia (artificially prolonged PT) →
APS (false positive) –>

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5
Q

Differential for prolonged PTT in patient with abnormal bleeding and in patient with no bleeding history

A

ABNORMAL:
Acquired VWS →
Factor 8, 9, or 11 deficiency →
Factor 8, 9, or 11 inhibitor
NO bleeding:
Factor XII
Lupus anticoagulant

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6
Q

Differential for prolonged PTT + PT

A

severe liver disease
DIC
*severe vitamin k deficiency
*common pathway defects – factor X deficiency or inhibitor + factor V inhibitor

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7
Q

Perioperative management of factor VIII heterozygote

A
  • check baseline factor level
  • if in normal range, no prophylactic therapy
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8
Q

Enzyme that affects Xa inhibitor concentrations (drug interactions)

A

CYP3A4

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9
Q

enzyme affecting wafarin concentrations

A

CYP2C9

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10
Q

Conditions associated with acquired VWS

A

MPN →
Plasma cell dyscrasias ->
Lymphoproliferative disorder →

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11
Q

Intrinsic pathway measured by what

A

PTT

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12
Q

What is serum?

A

Plasma minus clotting factors

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13
Q

Do coagulation tests use plasma or serum? What are collection tubes manufactured to contain anticoagulants?

A

1) Plasma. Plasma is unclotted blood (separated from buffy coat and RBCs) so contains clotting factors. Can’t use serum because all the clotting factors have been used up.
2) Preserve clotting factors

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14
Q

Why is it referred to as extrinsic pathway?

A

Activated by an extrinsic factor (eg tissue factor)

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15
Q

Utility of INR

A
  • primary intended for titrating warfarin
  • It has no predictive value for bleeding risk in patients not on warfarin per Dr. Shatzel
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16
Q

Function of thrombin

A

Converts fibrinogen to fibrin

17
Q

Why measure thrombin time?

A

1) Test for fibrinogen deficiency or dysfunction
2) Detect whether thrombin inhibiting drug is present in plasma (dabigatran, argatroban, bivalrudin)

18
Q

Prekallikrein deficiency presentation

A

No bleeding diasthesis, similar to factor XII

19
Q

Factor XI (hemophilia c) presentation

A

Mild to almost non existent bleeding diasthesis

20
Q
A