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Heme and Lymph Final > DSA: Thrombosis and Hemostasis > Flashcards

DSA: Thrombosis and Hemostasis Flashcards

1
Q

What is platelet adhesion mediated by?

A

vWF

  • it sticks circulating platelets to the area of damaged vessel wall
  • binds to platelet membrane glycoprotein 1b
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2
Q

What do platelets release when they adhere to the damaged endothelium?

A

-ADP
-TXA2
-

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3
Q

What cleaves prothrombin into thrombin?

A

activated Xa

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4
Q

What are the physiologic antithrombotic systems that inhibit platelet activation?

A
  • aniththrombin III
  • Ptn C and S
  • tissue factor pathway inhibitor (TFPI)
  • the fibrinolytic system
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5
Q

What does the ptn C and Ptn S do?

A

destroy factor Va and VIIIa

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6
Q

What does the PT measure

A

the integrity of the etrinsic and common pathway sof coagulation

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7
Q

What does PTT measure?

A

the integrity of the intrinsic and common pathways of coagulations

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8
Q

What is TT?

A

a screen for quantitative deficiencies and qualitative defects of plasma fibrinogen

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9
Q

What does it mean if we have a pronlonged PT or PTT?

A

-deficiency in one or more coagulation factors or the presence of an inhibitor (usually antibody)

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10
Q

Why do we do that weird 1:1 test?

A

if the PT or PTT is corrected, that confirms that something was missing in the person’s serum
-we mixed it with normal serum

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11
Q

what if the 1:1 test does correct the PTT and PT?

A

we need to assay for specific coagulation factors

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12
Q

What if the 1:1 test doesn’t correct the PT PTT values?

A

and inhibitor is probably there in the patient’s plasma

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13
Q

What does prolonged TT mean?

A

quantitative deficiency or qualitative defect of fibrinogen

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14
Q

In a preop patient, do we automatically want to run the coagulation tests on them?

A

no, not unless they have a bad history

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15
Q

How is a venous thromboembolism often precipitated?

A

by the combo of an underlying hypercoagulable genotype and an acquired prothrombotic, hypercoagulable state such as pregnancy, immobilization, or the postoperative state

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16
Q

What are patients who present with VTE at increased risk for?

A

an occult malignancy

-occult= not accompanied by readily discernable signs and symptoms

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17
Q

What is spontaneous miscarriages characteristic of?

A

antiphospholipid syndrome

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18
Q

inheritance of vW disease

A

auto dominant

-remember hemophilia is X-linked recessive

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19
Q

What happens in VWD?

A

VWF is decreased

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20
Q

What does vWF normally do?

A
  • mediates platelet adhesion and aggregation

- carries and stabilizes factor VIII

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21
Q

What is the stored reservoir of multimerized VWF in vascular endothlial cells?

A

Weibel-Palade bodies

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22
Q

What normally cleaves VWF?

A

ADAMTS13

-deficiency of this is TTP

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23
Q

Type 1 and 3 VWD

A

quantitative

-3 is virtually absent

24
Q

Type 2 VWD?

A

qualitative defect

25
Q

What is more rapidly cleared from the blood in VWD (2nN)?

A

factor 8

-the protein on vWF that binds it is screwed up

26
Q

How does VWD present?

A

they bleed a lot

27
Q

What labs are recommended for initial evaluation if you think someone might have VWD?*

A
  • VWF Ag
  • ristocetin cofactor activity
  • factor VIII coagulant activity
28
Q

What 3 things do we need to do when managing someone with VWD?

A
  • increase plasma concentration of VWF and FCIII
  • replace or supplement VWF and FVIII by using human plasma-derived, viral-inactivated concentrates
  • promote hemostasis using hemostatic agents that dont use the VWF mechanism
29
Q

What do we suspect if someone has abnormal VWF test results and bleeding symptoms without a personal or family history consistent with hereditary VWD?

A

Acquired von Willebrand syndrome

-AVWS

30
Q

Bernard-Soulier syndrome and Glanzmann Thrombasthenia

A
  • BSS results from deficiency of platelet membrane glypoprotein Ib-IX-V complex that is the principal receptor for binding VWF… platelets can’t adhere to vessels now
  • GT: deficiency of platelet GPIIb-IIIa complex that is the principal receptor for binding plasma fibrinogen…. platelets can’t aggregate now
31
Q

Which disorder has enlarged platelets?

A

BSS

-platelets are normal in GT

32
Q

If we add ristocetin and we have BSS, what will happen?

A

nothing

  • means it’s BSS
  • GT has normal response to ristocetin, but don’t respond to anything else
33
Q

Platelet storage pool disorders

-platelet dense granule storage pool deficiency (DG-SPD)

A

-HErmansky Pudlak syndrome: ocluocutaneous albinism

34
Q

DG-SPD with Wiskott Aldrich

A

associated with microthrombocytopenia

35
Q

DG-SPD with Chediak-Higashi syndrome

A

partial albinism and leukocyte inclusions

36
Q

gray platelet syndrome

A
  • auto recessive
  • DG-SPD
  • enlarged platelets that are gray
37
Q

Hereditary hemorrhagic telangiectasia

-Osler weber rendu syndrome

A
  • auto domninant
  • severe recurring nosebleeds and GI bleeds
  • resulting in chronic anemia nd iron deficiency
  • HHT genes mutated
  • bevacizumab normalizes cardiac index
38
Q

Ehlers-Danhlos syndrome

A
  • mutations in genes encoding fibrillar collagen
  • auto dominant
  • skin or join hyperextensibility
  • bleeding tendency and easy bruisability
39
Q

Henoch-Schonlein purpura

A
  • children
  • rash on legs
  • arthralgias
  • abdominal pain
  • deposition of IgA immune complexes in the skin, GI tract, and kidneys
40
Q

Antithrombin III deficiency

A
  • accumulation of fibrin

- lifelong propensity to thrombosis

41
Q

What 3 things are the hypercoagulable states associated with a deficiency of antithrombotic factors?

A
  • Antithrombin III deficiency
  • Protein C deficiency
  • Protein S deficiency
42
Q

What things are associated with increased prothrombotic factors?

A
  • Factor Va (Leiden, ptn C resistance)
  • Prothrombin (G20210A mutation)
  • Factor IX Padua
43
Q

Protein C deficiency

A

-leads to unregulated fibrin generation because of impaired incactivation of factors VIIIa and Va,
-auto dominant
-

44
Q

Protein S deficiency

A
  • principal cofactor of activated protein C
  • looks just like protein C when it’s gone
  • no regulation of fibrin generation
45
Q

Activated Protein C Resistance (Factor V Leiden)

A
  • frequent in european ancestry
  • point mutation for factor V
  • Arg to Gln mutation renders factor Va incapable of being inactivated by APC
46
Q

Prothrombin Gene Mutation (prothrombin G20210A)

A
  • sub G for A in prothrombin gene
  • elevated levels of prothrombin
  • increased risk for venous thrombosis
47
Q

What do Protein S and C depend on for their activity?

A

Vitamin K!

-so warfarin is a Vit K antagonist and really screws us over… warfarin induced skin necrosis

48
Q

should you test someone for a primary hypercoagulable state immediately after a major thrombotic eve?

A

no

  • all the antithrombotic proteins are consumed
  • leads to erroneous diagnosis of inherited antithrombin, Ptn C, or Ptn S deficiency
49
Q

hyperhomocysteinemia

A
  • premature atherosclerotic disease and VTE

- can cause vascular endothelial injury

50
Q

What kind of cancer is hypercoagulability most prominent in?

A

panreatic cancer

51
Q

What are the most frequent thrombotic manifestations in patients with neoplasms?

A

DVT and PE

52
Q

What is highly associated with adenocarcinomas?

A

Trousseau’s syndrome and nonbaterial thrombotic endocarditis

53
Q

Antiphospholipid syndrome

A
  • venous and arterial thrombosis
  • recurrent spontaneous preggo loss
  • thrombocytopenia
54
Q

Hereditary Hemorrhagic telangiectasia EOD

-Osler-Weber-Rendu syndrome

A
  • recurrent epistaxis
  • Mucocutaneous telangiectases
  • Visceral arteriovenous malformations (especially lung, liver, brain, bowel)
55
Q

Genetics of HHT?

A
  • Auto dominant

- ENG, ALK1, SMAD4

56
Q

What does HHT look like?

A
  • they bleed a lot

- AVMs

57
Q

Tx of HHT?

A
  • if you think it’s there, do an MRI of the brain with contrast
  • if AVM 1-2 mm in diameter, do embolization
  • after that, do CT angiogram in 6 months and 3 years

Heme and Lymph Final (14 decks)

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