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Unit 4: Blood and Lymph > Pathoma: 2a hemostatsis and related disorders > Flashcards

Pathoma: 2a hemostatsis and related disorders Flashcards

1
Q

What is the goal of 2a hemostasis?

A

to stabilize the platelet plug

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2
Q

How does 2a hemostasis achieve its end goal?

A

via the coagulation cascade

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3
Q

What is the receptor for platelet aggregation?

A

GPIIb/IIIa

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4
Q

What molecule links the platelets via GPIIb/IIIa?

A

fibrin

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5
Q

What is the end product of the coagulation cascade?

A

thrombin

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6
Q

What does thrombin do?

A

it converts fibrinogen to fibrin

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7
Q

What gets crosslinked to generate a stable platelet plug?

A

fibrin

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8
Q

What 3 things does activation require?

A
  1. exposure to an activating surface
  2. phospholipid surface
  3. calcium
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9
Q

Where does the calcium come from?

A

dense platelet granules

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10
Q

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

A

factor abnormalities

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11
Q

______ is the major clinical feature of 2a hemostasis.

A

Deep bleeding (into muscles and joints) and rebleeding after surgery

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12
Q

In 1a hemostasis, the primary problem is ______.

A

superficial bleeding (into mucosa and

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13
Q

What is the PT test?

A

a measure of extrinsic and common factors

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14
Q

What is the PTT test?

A

a measure of intrinsic and common factors

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15
Q

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

A

12, 11, 9, 8, 10

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16
Q

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

A

7, 10

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17
Q

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

A

10, 5, 2, 1

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18
Q

What activates factor 12 (the intrinsic pathway)?

A 
subendothelial collagen (SEC)
* PTT and SEC are both 3 letters
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19
Q

What activates factor 7 (the extrinsic pathway)?

A 
tissue thromboplastin (TT)
* TT and PT are both 2 letters
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20
Q

Which test is better for measuring heparin?

A

PTT

* PTT and HEP are both 3 letters

21
Q

Which test is better for measuring warfarin/Coumadin?

A

PT

22
Q

What is hemophilia A?

A

an X-linked defect of factor VIII (8)

* hemophilia AAAAA-eight

23
Q

How do hemophilia A pts present?

A

deep tissue, joint, and post-surg bleeding

24
Q

The clinical severity of hemophilia A depends on _____.

A

the degree of deficiency

25
Q

Hemophilia A lab results?

A 
increased PTT
normal PT
decreased FVIII
normal platelets
normal bleeding time
26
Q

What is the treatment for Hemophilia A?

A

give recombinant factor VIII

27
Q

What is Hemophilia B?

A

a genetic factor IX (9) deficiency

28
Q

What is another name for Hemophilia B?

A

Christmas disease

29
Q

What is a coagulation factor inhibitor?

A

an autoantibody against a coagulation factor, impairing its function

30
Q

Which factor is commonly targeted by coagulation factor inhibitors?

A

factor VIII (8)

31
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

32
Q

What is Von Willebrand disease?

A

the most common inherited coagulation disorder- deficiency of vWF

33
Q

What is the most common subtype of vW disease?

A

auto. dominant; decreased vWF levels

34
Q

How does a vW Disease pt present?

A

mild mucosal and skin bleeding

35
Q

What does low vWF cause?

A

impaired platelet adhesion

36
Q

What are the lab findings in vW Disease?

A

increased bleeding time
increased PTT
normal PT
abnormal ristocetin test

37
Q

Why is the PTT increased in vW Disease?

A

vWF stabilizes factor VIII (8)

38
Q

What is the ristocetin test?

A

ristocetin given to platelets makes them aggregate- if there’s no vWF, they won’t clump

39
Q

What is the tx for vW Disease ?

A

desmopressin

40
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

41
Q

What is Vitamin K deficiency?

A

disrupted coagulation

42
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

43
Q

Vitamin K is activated by ____ in the ____.

A

epoxide reductase; liver

44
Q

_____ blocks epoxide reductase.

A

Coumadin

45
Q

When can vitamin K deficiency present?

A
  1. newborns
  2. long-term Abx
  3. malabsorption (esp fat)
  4. liver failure
46
Q

Why does long term Abx therapy cause Vitamin K deficiencies?

A

vit K is synthesized by normal gut flora

47
Q

How is vitamin K deficiency prevented in newborns?

A

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

48
Q

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

A

PT

49
Q

What happens when large volume transfusions are given?

A

coagulation factors are diluted, leading to deficiencies

Unit 4: Blood and Lymph (25 decks)

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