Blood Disorders-Assessment Exam 3 Flashcards

1
Q

_____ plays a critical role in platelet adherence/adhesion

A

vWF

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2
Q

What is the most common hereditary bleeding disorder?

A

vWF

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3
Q

Common s/s of vWF

A
  • Easy bruising
  • Recurrent epistaxis
  • Menorrhagia

Pts usually unaware until questionnaire/surgery

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4
Q

Classification of inherited vWF disease

A

Know Type 1 is most mild, type 3 is severe but also rare

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5
Q

Diagnostics for vWF

A

PT/aPTT usually normal
- bleeding time is prolonged
- Need Hematologist

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6
Q

What type of blood products do we usually give vWF?

A

Factor 8, cryo, specific factors for pt

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7
Q

Treatment for vWF

A

Correct the deficiency of vWF
• Using desmopressin
• By the transfusion of the specific factor
• Cryoprecipitate

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8
Q

What is the dose for DDAVP for vWF?

A

.3 mcg/kg - .8 mcg/kg

In 50 ml of NS over 15-20 min

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9
Q

What is DDAVP?

A

Basically synthetic vasopressin: stimulates the release of vWF by endothelial cells

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10
Q

What is the maximal effect and duration of time for DDAVP?

A

30 minutes
6-8 hrs

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11
Q

Side effects of DDAVP

What is the biggest one?

A

Hyponatremia is the biggest

Headache, rubor, hypotension, tachycardia, and water intoxication

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12
Q

In order to decrease water intoxication, hyponatremia, and consequent seizures, the administration of _____, orally or intravenously, should be restricted for _______ hours after the use of the drug

A

Water
4-6 hrs

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13
Q

Hyponatremia s/s chart

A
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14
Q

In common preparation, the cryoprecipitate is not submitted to _________ and, therefore, poses an increased risk of _______

A

Viral attenuation
Infection

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15
Q

What can you use for vWF if unresponsive to DDAVP?

A

Cryoprecipitate

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16
Q

____ unit of cryo raises fibrinogen levels by _____ mg/dL

A

1 unit
50 mg/dL

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17
Q

How is factor VIII concentrate prepared?

A

from the pool of plasma from a large number of donors and undergoes viral attenuation

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18
Q

What does factor VIII contain?

A

factor VIII (obviously)
vWF

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19
Q

When is factor VIII commonly given?

A

Pre op
During surgery

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20
Q

What are anesthesia considerations for vWF?

A
  • Prior evaluation by a hematologist
  • When indicated, DDAVP should be infused 60 minutes before the surgery
  • Normalization of the bleeding time and improved levels of F VIII should be confirmed before the surgery in patients
  • Patients with coagulopathies undergoing neuroaxial block = increased risk of developing a hematoma and compression of neurological structures
  • Avoid traumas during the anesthesia
  • Arterial puncture is not recommended
  • Laryngeal trauma during tracheal intubation may cause hematoma = postoperative obstruction of the airways
  • IM avoided
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21
Q

What is acquired bleeding?

A

Bleeding from:
- heparin
- warfarin
- fibrinolytic
-antiplatelets

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22
Q

Heparin molecular set up

A

Negatively charge, CHO containing glucuronic acid residues

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23
Q

What are 2 types of heparin?

A

Unfractionated
LMWH

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24
Q

Heparin inhibits _____

A

Thrombin

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25
What is thrombin used for?
Converting fibrinogen to fibrin
26
Heparin derives it anticoagulant effect by activating _________
antithrombin III
27
What labs do you get when pt is on heparin?
aPTT, ACT
28
What is heparin rapidly reversed by? How?
Protamine + polypeptide forming a stable complex neutralizing heparin
29
LMWH is more effective at _______ compared to UFH
VTE prophylaxis
30
LMWHs have a more predictable ___________, fewer effects on __________, and a reduced risk for _________.
pharmacokinetic response platelet function heparin-induced thrombocytopenia (HIT)
31
T/F Monitoring of LMWH is performed routinely
False
32
What does warfarin interfere with? What coagulation factors are dependent on this (the thing we are interfering)?
hepatic synthesis of vitamin K Factors II, VII, IX, X
33
What is the normal/slower way to reverse warfarin? How fast does it take to correct?
Vitamin K 6-8 hrs
34
What is the more rapid reversal of warfarin?
prothrombin complex concentrates, recombinant factor VIIa and FFP
35
How do fibrinolytics act?
By converting plasminogen to plasmin, which in turn cleaves fibrin, thereby causing clot dissolution
36
Examples of fibrinolytics
Tissue plasminogen activator (tPA), streptokinase (SK), and urokinase (UK)
37
What are antifibrinolytic agents? What do these do?
tranexamic acid, ε–aminocaproic acid, and aprotinin inhibits the conversion of plasminogen to plasmin
38
Consideration for antiplatelets
- D/c drugs on time - platelet transfusion
39
What is DIC?
Disseminated Intravascular Coagulopathy - Systemic activation of the coagulation system simultaneously leads to thrombus formation and exhaustion of platelets and coagulation factors
40
What underlying disorders may precipitate DIC?
trauma, amniotic fluid embolus, malignancy, sepsis, or incompatible blood transfusions
41
DIC pathway picture
42
What will labs look like in DIC?
- Reductions in PLT - prolongation PT, PTT, and thrombin time (TT) - elevated concentrations of soluble fibrin degradation products
43
Management of DIC requires alleviating the ________ condition precipitating ________ activation
Underlying Hemostatic
44
Treatment for DIC includes:
blood component transfusions to replete coagulation factors and platelets consumed in the process
45
What is generally contraindicated in DIC? Why?
Anti-fibrinolytics Potential for catastrophic thrombotic complications
46
What are prothrombotic disorders?
Factor V Leiden HIT
47
What is factor V used for?
Protein for normal clotting - When enough fibrin has been made, a substance called activated protein C inactivates factor V, helping stop the clot from growing any larger than necessary
48
What is factor V Leiden?
Mutations of the genes for factor V - abnormal version of factor V that is resistant to the action of activated protein C
49
In factor V leiden, is factor V responsive to activated protein C?
No! It cannot easily stop factor V from making more fibrin (making more clots)
50
Factor V Leiden is associated with an increased risk of developing an episode of ________ (with or without a ____)
DVT PE
51
Do you usually put pts with factor V Leiden on anticoagulants? What about in pregnancy? Why?
Yes to both - to prevent venous or placental thrombosis, because improved placental blood flow is likely to lead to better pregnancy outcomes
52
Most factor V Leiden are silent until ______
Pregnancy
53
What are the first presentations of factor V Leiden?
DVT repeated missed abortions recurrent late fetal losses
54
Common anticoagulation therapies for factor V Leiden?
warfarin unfractionated heparin LMWH
55
HIT describes an _________ drug reaction occurring in as many as ____% of patients after exposure to unfractionated heparin or (rare cases) LMWH
Autoimmune-mediated 5%
56
When does thrombocytopenia occur in HIT?
5-14 days after initial therapy
57
What is the hallmark finding for HIT?
decrease in PLT < 100,000
58
HIT results in _____ activation and potential for _________
Platelet Venous/arterial thromboses
59
Evidence suggests that HIT is mediated by what immune complexes?
composed of IgG antibody, platelet factor 4 [PF4], and heparin
60
Patients developing HIT during heparin therapy experience substantially increased risk for ________ (absolute risk ____%-____%)
Thrombosis 30-75
61
HIT pathway picture
62
A diagnosis of HIT should be entertained for any patient experiencing what?
thrombosis or thrombocytopenia during or after heparin administration
63
What is one of the most important things to do in suspected HIT cases?
D/C heparin STAT - including unfractionated heparin, heparin-bonded catheters, heparin flushes, LMWH
64
Alternative _____ _______ must be administered concurrently in HIT
non-heparin anticoagulation
65
What is usually substituted for heparin in HIT? What are examples of this drug?
direct thrombin inhibitor - bivalirudin, lepirudin, argatroban
66
Wha can you use to treat VTE in HIT? What is the class of this drug?
Fondaparinux - synthetic Factor Xa inhibitor
67
Typically, PF4/heparin immune complexes clear from the circulation within _____ months
3
68
How do you treat anti-thrombin 3 deficiency?
Platelets
69
TXA dose for adult and peds
1-2 grams for adults 15 mg/kg peds
70
What is a weird side effect of TXA?
TXA toxicity - they can lose color vision
71
What is the apache score?
Used to evaluate pts with DIC and sepsis