Spring 2024 (Exam II) Blood Disorders Flashcards

1
Q

What is the number 1 most common hereditary bleeding disorder?

A

vWF disorder (platelet adhesion dysfunction)

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2
Q

What are the S/S of vWF disorder?

A
  • Easy bruising
  • epistaxis
  • menorrhagia (menstrual bleeding)
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3
Q

What would lab values be for someone with vWF deficiency?

A
  • Normal PT & aPTT
  • Bleeding time is prolonged
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4
Q

What are the treatments for vWF deficiency?

A
  • Desmopressin
  • Cryoprecipitate
  • Factor VIII
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5
Q

How does DDAVP work in regards to treatment of von Willebrand deficiency??

A

Stimulates vWF release from endothelial cells

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6
Q

What is the dose for DDAVP?

A

0.3 mcg/kg in 50 mL over 15-20 mins (Do not bolus)

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7
Q

What is the onset & duration of DDAVP?

A
  • Onset: 30mins (max effect)
  • Duration: 6-8hrs

Don’t bolus DDAVP; drip slowly.

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8
Q

What are the side effects of DDAVP?

A
  • HA
  • Stupor
  • hypotension (if given quickly)
  • tachycardia
  • hyponatremia (d/t retention of water, pt needs to be on fluid restriction)
  • water intoxication(excessive water retention)
    • restict water PO/ IV
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9
Q

What is the major side effect of DDAVP?

A

Hyponatremia

Na+ 120: Confusion, restlessness, wide QRS
Na+ 115: Somnolence, nausea, elevated ST, wide QRS
Na+ 110 Seizure, coma, V-tach, V-fib

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10
Q

Someone that gets DDAVP needs to be on what?

A

Fluid restriction 4-6hrs before & after DDAVP

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11
Q

What blood product can be utilized for vWF disease if the patient is unresponsive to DDAVP?

A

Cryoprecipitate

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12
Q

1 unit of Cryo raises the ____ level by ___?

A

Fibrinogen by 50 mg/dL

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13
Q

What is a potential risk factor with cryoprecipitate?

A

Increased risk of infection (not submitted to viral attenuation/ multi-pool donors)

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14
Q

What is Factor VIII concentrate made of?

A

Pool of plasma from a large number of donors.
Contains Factor VIII and vWF

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15
Q

When is Factor VIII concentrate given?

A

Preop or intraop
* contains F VIII and vWF

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16
Q

Between Cryo and F VIII concentrate, which has a higher risk for infection? Why?

A
  • Cryo
  • multi-pool donors with no viral attenuation
  • F VIII concentrate does undergo viral attenuation
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17
Q

Pts with coagulopathies undergoing neuraxial anesthesia are at increased risk for what?

A
  • Hematoma
  • Nerve compression
18
Q

What are the anesthesia considerations for someone with vWF deficiency?

A
  • Avoid trauma (Get the best intubator performing DL)
  • Laryngeal trauma during tracheal intubaton may cause hematoma= postop obstruction of airway
  • avoid IM sticks (Will cause localized tissue trauma)
  • avoid arterial lines (if feasible)
  • avoid spinals
19
Q

How does heparin work?

A
  • Heparin inhibits thrombin by activating antithrombin III
  • AT-III will inhibit thrombin
20
Q

What labs are monitored with heparin?

A

PTT &/or ACT

21
Q

Use and benefits of LMWHs

A
  • VTE prophylaxis
  • more predictable pharmacokinetic response
  • fewer effects on plt function
  • reduced risk for HIT
  • no labs monitoring
22
Q

What is the mechanism of action of Coumadin?

A

Inhibition of vitamin K-dependent factors

23
Q

Which factors are vitamin-K dependent?

A

II, VII, IX & X

24
Q

What is the onset for Vitamin K?

A

6-8hrs

25
Q

What drugs/products can be given to reverse coumadin faster than Vit K?

A
  • Prothrombin complex concentrates
  • Factor VIIa
  • FFP
26
Q

What is the mechanism of action for fibrinolytics (UK, streptokinase & tPA)?

A

Convert plasminogen to plasmin, which cleaves fibrin

27
Q

How do tranexamic acid (TXA) and aminocaproic acid work?

A

Inhibit conversion of plasminogen to plasmin

28
Q

What is the best way to treat DIC?

A

Treat the underlying cause

29
Q

What will labs show for someone in DIC?

A
  • ↓PLTs
  • Prolonged PT, PTT & thrombin time
  • ↑ fibrin degradation products
30
Q

When is antifibrinolytic therapy given to someone in DIC?

A

Trick question, it shouldn’t. Can lead to catastrophic thrombotic complications

31
Q

What is factor V Leiden?

A
  • Protein for clotting.

Activated protein C inactivates factor V thus stopping clot growth (negative feedback).

32
Q

What is Factor V Leiden deficiency?

A

Genetic mutation where Activated protein C cannot stop factor V Leiden thus excessive fibrin.

33
Q

What does Activated Protein C do?

A

Inactivates factor V when enough fibrin has been made.

34
Q

Factor V Leiden increases risk of ?

A

DVT w/ wo PE

35
Q

Who is usually tested for Factor V Leiden?

A

Pregnant women. Especially ones with unexplained late stage miscarriages

36
Q

What anticoagulant medications could a pregnant woman with Factor V Leiden be put on?

A
  • LMWH
  • For individuals with severe Factor V, they will be placed on Coumadin
37
Q

What is the hallmark sign of HIT?

A

Plt count <100,000

Thrombocytopenia occurs 5-14 days after initial heparin therapy and affects 5% of patients exposed to unfractionated heparin and rare cases LMWH.

38
Q

HIT results in ____ activation and potential____?

A

platelet; thrombosis

39
Q

What is heparin replaced with when HIT is diagnosed?

A

Agratroban or bivalirudin, lepirudin, argatroban (direct-thrombin inhibitors)

40
Q

What is Fonaparinaux & when is it used?

A
  • A synthetic Factor Xa inhibitor
  • used to treat VTE in HIT