Blood: 4.2: 2a hemostasis Flashcards

1
Q

What does thrombin do?

A

it converts fibrinogen to fibrin

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2
Q

What is the ristocetin test?

A
  • ristocetin + platelets –> aggregation
  • if there’s no vWF, they won’t clump
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3
Q

In 1a hemostasis, the primary problem is ______.

A

superficial bleeding

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4
Q

What test would check the effect of liver failure on coagulation?

A

PT

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5
Q

______ and _______ are the major clinical feature of 2a hemostasis.

A
  • Deep bleeding (into muscles and joints)
  • rebleeding after surgery
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6
Q

What is the PTT test?

A
  • a measure of intrinsic and common pathways
    • PTT = more letters than PT, so it’s the LONGER pathway
    • 12, 11, 9, 8, 10, 5, 2, 1
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7
Q

What is the most common subtype of vWF disease? What is the result?

A
  • auto. dominant
  • decreased vWF levels
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8
Q

What is Vitamin K deficiency?

A

disrupted coagulation

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9
Q

What does low vWF cause?

A

impaired platelet adhesion

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10
Q

Why is Vitamin K so important for coagulation?

A

it’s necessary for gamma-carboxylation of factors 2, 7, 9, 10, C, and S

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11
Q

Platelet activation requires calcium. Where does the calcium come from?

A

dense platelet granules

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12
Q

How does desmopressin work?

A
  • it increases vWF release from the Weibel-Palade bodies in endothelial cells
  • * W for vWF, P for P-selectin
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13
Q

The clinical severity of hemophilia A depends on _____.

A

the degree of deficiency

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14
Q

Where does Vitamin K come from?

A

normal gut flora

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15
Q

Platelets adhere using vWF and _____.

A

GPIb

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16
Q

How do hemophilia A pts present?

A
  • deep tissue, joint, and post-surg bleeding
    • these are classic s/s of a secondary hemostasis disorder
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17
Q

Hemophilia A lab results?

A
  • increased PTT
  • normal PT
  • decreased FVIII
  • normal platelets
  • normal bleeding time
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18
Q

Which factor is most commonly targeted by coagulation factor inhibitors?

A

factor VIII (8)

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19
Q

What gets cross-linked to generate a stable platelet plug?

A

fibrin

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20
Q

Why does long term Abx therapy cause Vitamin K deficiencies?

A
  • vit K is synthesized by normal gut flora
  • loss of gut bacteria = loss of vitK
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21
Q

What is the treatment for Hemophilia A?

A
  • give recombinant factor VIII
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22
Q

What is another name for Hemophilia B?

A

Christmas disease

23
Q

What molecule links the platelets via GPIIb/IIIa?

A

fibrinogen

24
Q

What happens when large volume transfusions are given?

A

coagulation factors are diluted, leading to deficiencies

25
Q

What is the PT test?

A
  • a measure of extrinsic and common pathways
  • PT is SHORTER than PTT, so PT is less factors
  • factor 7, 10, 5, 2, 1
26
Q

Where are the coag. cascade factors produced?

A

in the liver

27
Q

_____ blocks epoxide reductase.

A

Warfarin/Coumadin

28
Q

What is hemophilia A?

A
  • an X-linked recessive defect of factor VIII (8)
    • can be de-novo mutation
  • * hemophilia AAAAA-eight
29
Q

In which pts can vitamin K deficiency present?

A
  1. newborns
  2. long-term Abx users
  3. malabsorption (bc it’s a fat soluble vitamin)
  4. liver failure (less production of factors and they’re not activated bc of loss of epoxide reductase)
30
Q

What is the goal of 2a hemostasis?

A

to stabilize the weak platelet plug

31
Q

What are the lab findings in vWF disease?

A
  • increased bleeding time
  • increased PTT (bc vWF stabilizes factor VIII)
  • normal PT
  • abnormal ristocetin test
32
Q

What activates factor 12 (the intrinsic pathway)?

A
  • subendothelial collagen (SEC)
  • * PTT and SEC are both 3 letters
33
Q

What is Hemophilia B?

A
  • a genetic factor IX (9) deficiency
  • resembles hemophilia A
34
Q

What is the order of the common pathway, starting at 10?

A
  • 10, 5, 2, 1
    • “1 x 2 x 5 = 10”
35
Q

What is a coagulation factor inhibitor? Which is most common?

A
  • an acquired autoantibody against a coagulation factor, impairing its function
  • anti-FVIII (factor 8) most common
36
Q

What is the receptor for platelet aggregation?

A

GPIIb/IIIa

37
Q

Which test is better for measuring warfarin/Coumadin?

A
  • PT/INR
38
Q

What 3 things does activation require?

A
  1. exposure to an activating surface
  2. phospholipid surface
  3. calcium
39
Q

The goal of the coag. cascade is to produce factor ____.

A

X

“produce a perfect 10”

40
Q

Why is the PTT increased in vWF Disease?

A

vWF stabilizes factor VIII (8)

41
Q

Vitamin K is activated by ____ in the ____.

A
  • epoxide reductase
  • liver
42
Q

Activation requires a phospholipid surface. Where does this come from?

A

the surface of the platelets

43
Q

How does a pt with vWF disease present?

A

mild mucosal and skin bleeding

44
Q

Clinically, how can you tell the difference btw Hemophilia A and a coagulation factor inhibitor against factor VIII?

A

A mixing study: the increased PTT can be corrected in Hemophilia A by mixing pt’s plasma with a normal plasma, but this can’t happen if an inhibitor is present

45
Q

What is the order of the intrinsic pathway factors, ending at 10?

A

12 –> 11 –> 9 –> 8 –> 10

46
Q

In general, a disorder of 2a hemostasis is due to _____.

A

factor abnormalities

47
Q

How does 2a hemostasis achieve its end goal?

A

via the coagulation cascade

48
Q

What is Von Willebrand disease?

A
  • a deficiency of vWF
  • the most common inherited coagulation disorder
49
Q

What activates factor 7 (the extrinsic pathway)?

A
  • tissue thromboplastin (TT)
  • * TT and PT are both 2 letters
50
Q

What is the order of the intrinsic pathway factors, ending at 10?

A

7, 10

51
Q

How is vitamin K deficiency prevented in newborns?

A

they’re all given a prophylactic vitamin K shot at birth

52
Q

What is the end product of the coagulation cascade?

A

thrombin

53
Q

Which test is better for measuring heparin?

A
  • PTT
  • * PTT and HEP are both 3 letters
54
Q

What is the tx for vW Disease ?

A

desmopressin