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Heme Onc Royal College > Hemostasis / Thrombosis > Flashcards

Hemostasis / Thrombosis Flashcards

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1
Q

which clotting factor deficiency is not associated with a bleeding tendency?

A

factor XII (12)

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2
Q

What 2 factors associated with severe bleeding in heterozygous state?

A

FXI and FVII deficiencies

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3
Q

What is the treatment for FV & fibrinolytic factor deficiencies?

A

FFP

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4
Q

What is the treatment of FII and X deficiencies?

A

Prothrombin complex concentrates(PCC)

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5
Q

What is the treatment of FXIII and fibrinogen disorders?

A

Purified concentrates(FXIII and fibrinogen) or cryoprecipitate when concentrates not available

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6
Q

What happen to fibrinogen when both PT and aPTT are prolonged?

A

Fibrinogen will be reduced or abnormal

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7
Q

When thrombin time prolonged?

A

When fibrinogen is reduced or abnormal, in presence of inhibitors, presence of thrombin inhibiting drugs and hypoalbuminemia.

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8
Q

What clotting factor deficiencies suspected if aPTT normalized in mixing studies?

A

FXII, FXI, FIX & FVIII

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9
Q

If PT normalized in mixing study what factor deficiencies?

A

FVII

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10
Q

If both PT and aPTT normalized in mixing studies?

A

FX, FV, FII and fibrinogen

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11
Q

What is the screening test for FXIII deficiency?

A

Urea clot lysis assay except in newborn period may give false positive result and mild deficiencies better to use functional FXIII assays ( FXIII activity, antigen and FXIII A and B subunit)

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12
Q

Where is vWF synthesized?

A

Megakaryocytes and endothelial cells

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13
Q

What is the important of desmopressin(DDAVP) for vWF?

A

It induce the release of vWF from storage sites into plasma

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14
Q

What bleeding disorder suspected with MSK bleed

A

Fibrinogen disorder, FX,FII and FXIII deficiencies

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15
Q

What bleeding disorder common with GI and CNS bleeds?

A

FX deficiency

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16
Q

What bleeding disorder suspected with umbilical cord bleed

A

Fibrinogen, FII, V, XIII deficiencies

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17
Q

virchow’s triad

A

stasis
endothelial damage
hypercoagulability

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18
Q

peds age groups at highest risk of thrombosis

A

newborns

adolescents

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19
Q

what is the cause of purpura fulminans?

A

homozygous deficiency of protein C and/or S

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20
Q

where are protein C and S made?

A

liver

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21
Q

what are the antiphospholipid antibodies?

A
  • lupus anticoagulant
  • anti-cardiolipin antibodies
  • anti-beta-2-glycoprotein I (beta2-GPI) antibodies
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22
Q

definition of antiphospholipid syndrome

A

The presence of thrombosis or pregnancy loss with the persistent (2 tests performed 12 weeks apart) presence of APLA (LAC or ACLA or B2-GPI)

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23
Q

what should you check in patients with MTHFR gene polymorphisms?

A

homocysteine level - if elevated, may be at increased risk of clot

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24
Q

who should have thrombophilia testing

A
  • unprovoked VTE
  • recurrent VTE
  • purpura fulminans
  • fam hx of thrombophilia (may provide anticipitory guidance about starting prothrombotic meds etc)
25
Q

management of a child who you suspect has purpura fulminans

A
  • test for protein C and S

- FFP 10-20ml/kg Q6-12 hr

26
Q

how to manage blocked CVC that needs removal

A

anticoagulation x 3-5 days prior to removal to minimize risk of embolization

27
Q

if a child presents with a PE, what should you look for?

A

DVT - 50% have extremity clot

28
Q

why does leukemia increase risk of thrombosis

A

increased thrombin generation

29
Q

what platelet counts are recommended for patients on anticoagulation?

A

> 50 for treatment

> 30 for prophylaxis

30
Q

three signs of RVT

A
  • thrombocytopenia
  • palpable flank mass
  • hematuria
31
Q

patients that may not respond to treatment with unfractionated heparin

A

those with antithrombin (FII) deficiency

32
Q

how does unfractionated heparin work

A

binds and potentiates the effect of anti-thrombin

33
Q

pathophysiology of warfarin induced skin necrosis

A

acquired protein c deficiency

34
Q

vitamin K dependent coagulation factors

A

factor 2, 7, 9, 10 and protein C

35
Q

UFH target anti-Xa levels

A
  1. 35-0.7 (aPTT 60-85 s)

- should use PTT when you have hyperbilirubinemia

36
Q

LMWH target anti-Xa levels

A

0.5-1.0

37
Q

target INR for mechanical valve

A

2.5-3.5

38
Q

treatment of DVT without risk factors

A

anticoagulate 9-12 months

39
Q

treatment of DVT with chronic risk factors

A

indefinite

40
Q

treatment of DVT with acute risk factors

A

x3 months, then switch to prophylaxis if risk factors still present

41
Q

what is warfarin induced skin necrosis

A

acquired protein C deficiency, occurs within 3-10 days of starting treatment with warfarin

42
Q

how can you test for HIT?

A

ELISA for anti-platelet factor 4 antibodies (high sensitivity, low specificity)

43
Q

components of the 4T score for HIT

A
  • degree of thrombocytopenia < 50%, plts > 20
  • timing of thrombocytopenia (5-10 days after starting heparin)
  • presence of new thrombosis
  • no other cause of thrombocytopenia
44
Q

diagnostic criteria for anti-phospholipid antibody syndrome

A

Defined by the presence of vascular thrombosis or pregnancy loss with the persistent (2 tests performed 12 weeks apart) presence of APLA (LAC or ACLA or B2-GPI)

45
Q

how do you test for a lupus anticoagulant? (3 steps)

A
  1. Demonstrating an abnormal aPTT
  2. Mixing study to show that it does not correct – due to presence of inhibitor
  3. Confirmatory test – add phospholipids to prove that the abnormality is phospholipid dependent
46
Q

what is the INR

A

(patient’s PT/control PT)to the power of ISI, where ISI = international sensitivity index (sensitivity of thromboplastin)

47
Q

list novel anticoagulants and their mechanism of action

A

Rivaroxaban/apixaban - oral factor Xa inhibitors

Dabigatran - oral direct thrombin inhibitor

48
Q

reversal of warfarin - INR > 8 and significant bleeding

A

FFP, Prothrombin complex concentrates, recombinant factor VIIa

49
Q

reversal of warfarin - INR > 8, no bleeding

A

vitamin K (PO or IV)

50
Q

on what chromosome is vWF encoded?

A

chromosome 12

51
Q

where is vWF made and stored?

A 
endothelial cells (stored in weibel-palade bodies)
and megakaryocytes (stored in alpha granules)
52
Q

functions of vWF

A
  • Platelet adhesion to injured endothelium via GPIb/IX and collagen
  • To assist with platelet aggregation via GPIIb/IIIa receptor
  • Carrier protein for F8 – protecting it from degradation by protein C
53
Q

inheritance of vWD

A

Type 1, Type 2 A/B/M - autosomal dominant

Type 2N, Type 3 - AR

54
Q

factors which affect vWF levels

A
  • ABO blood type (lower levels in Type O)
  • High estrogen states
  • Hypothyroidism (decreases levels of vWF)
  • Stress
  • Acute illness
  • DDAVP
55
Q

VWF/F8 concentrates

A

Humate-P
Willate
Alphanate

56
Q

Treatment options for VWD

A
  • antifibrinolytics
  • estrogens (OCP)
  • DDAVP (Risk of hyponatremia, seizures - tachyphylaxis)
  • VWF/factor 8 concentrates
  • cryo
57
Q

conditions associated with acquired VWD

A
  • Wilms tumour
  • Hypothyroid
  • SLE
  • Aortic stenosis
  • LVAD
58
Q

in what type of VWD is DDAVP contraindicated?

A

type 2B - can worsen thrombocytopenia

Heme Onc Royal College (30 decks)

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