Blood Disorders (Exam III)

Question 1

What are the S/S of vWF disorder? (3)

Answer 1

Easy bruising
epistaxis
menorrhagia

(patients are usually unaware until questionnaire/surgery)

Question 2

vWF plays a critical role in _______

Answer 2

Platelet adherence/adhesion

vWF Disorder..platelet COUNT is normal. It is the function that is messed up.

Question 3

Which type of vWF is the most mild and most common?

Answer 3

Type 1

Question 4

Which type of vWF is the most rare, most severe and usually requires factor concentrates?

Answer 4

Type 3

Question 5

Which types of vWF respond to DDAVP?

Answer 5

1
2A
2M
2B

(2N and 3 dont)

Question 6

Which vWF type is often confused with hemophilia A?

Answer 6

Type 2N

Question 7

What is the most common hereditary bleeding disorder?

Answer 7

vWF disorder

Question 8

What would PT, aPTT and bleeding time lab values be for someone with vWF deficiency?

Answer 8

• Normal PT & aPTT
• Bleeding time is prolonged

Question 9

What are the treatments for vWF deficiency?

Answer 9

• Desmopressin (DDAVP)
• Cryoprecipitate
• Factor VIII

Correction the deficiency of vWF

Question 10

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

Answer 10

Stimulates vWF release from endothelial cells

Question 11

DDAVP is a synthetic analogue of ______

Answer 11

Vasopressin

Question 12

What is the dose for DDAVP?

Answer 12

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

Question 13

What is the onset & duration of DDAVP?

Answer 13

• Onset: 30mins
• Duration: 6-8hrs

Question 14

What are side effects of DDAVP?

Answer 14

• HA
• Rubor
• hypotension
• tachycardia
• hyponatremia
• water intoxication (excessive water retention)

Question 15

What is the most major side effect of DDAVP?

Answer 15

Hyponatremia

Monitor Na levels!

Question 16

Describe why a patient on DDAVP would need to be on fluid restriction and when we would start/stop the restriction of fluids.

Answer 16

Fluid restriction 4-6hrs before & after DDAVP to decrease water intoxication, hyponatremia and seizure.

Question 17

What are the CNS changes and ECG changes for a Na level of 120.

Answer 17

CNS:
1. confusion
2. restlessness

ECG:
1. widening of QRS

Question 18

What are the CNS changes and ECG changes for a Na level of 115

Answer 18

CNS:
1. Somnolence
2. Nausea

ECG:
1. Elevated ST
2. Wide QRS

Question 19

What are the CNS changes and ECG changes for a Na level of 110.

Answer 19

CNS:
1. Seizures
2. Coma

ECG:
Vtach or Vfib

Question 20

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

Answer 20

Cryoprecipitate

Question 21

1 unit of Cryo raises the ____ level by ___?

Answer 21

Fibrinogen by 50 mg/dL

Question 22

What is a potential risk factor with cryoprecipitate?

Answer 22

Increased risk of infection (not submitted to viral attenuation)

Question 23

What is Factor VIII concentrate made of? What does it contain?

Answer 23

Pool of plasma from a large number of donors.

Contains F VIII and vWF

DOES undergo viral attenuation

Question 24

When is Factor VIII given?

Answer 24

Preop or intraop

Question 25

When should DDAVP be given prior to surgery?

Answer 25

60mins before

Question 26

True/False: Patients with coagulopathies should have surgery under general anesthesia

Answer 26

True

Question 27

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

Answer 27

- Hematoma - Nerve compression

Question 28

What are the anesthesia considerations for someone with vWF deficiency?

Answer 28

- Avoid trauma (particularly airway) - avoid IM sticks - avoid arterial lines (if feasible) - avoid spinals

Question 29

What are 4 types of meds that we give that can cause acquired bleeding?

Answer 29

1. Heparin 2. Warfarin 3. Fibrinolytic 4. Antiplatelets

Question 30

How does heparin work?

Answer 30

- Thrombin inhibition (thrombin needed to convert fibrinogen to fibrin) - Antithrombin III activation ## Footnote Heparin is negatively charged

Question 31

What labs are monitored with heparin?

Answer 31

PTT &/or ACT (how many seconds to clot)

Question 32

What is the antidote for heparin?

Answer 32

Protamine ## Footnote Protamine is positively charged polypeptide

Question 33

_____ is effective at VTE prophylasis compared to _____

Answer 33

LMWH; UFH

Question 34

which type of Heparin has a lower risk for HIT

Answer 34

LMWH

Question 35

What is the mechanism of action of Coumadin?

Answer 35

Inhibition of vitamin K-dependent factors.

Question 36

Which factors are vitamin-K dependent?

Answer 36

II, VII, IX & X (2, 7, 9, 10)

Question 37

What is the antidote for coumadin? what is the onset of this reversal?

Answer 37

Vitamin K. 6-8hrs

Question 38

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

Answer 38

- Prothrombin complex concentrates - Factor VIIa - FFP

Question 39

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

Answer 39

Clot dissolution: **Convert plasminogen to plasmin**, which cleaves fibrin

Question 40

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

Answer 40

**Inhibit conversion of plasminogen to plasmin**

Question 41

What is the **best** way to treat DIC?

Answer 41

Treat the underlying cause. Blood component transfusion to replete coag factors and plt used in the process

Question 42

What will labs show for someone in DIC?

Answer 42

- ↓Platelet count - Prolonged PT, PTT & Thrombin time. - ↑ fibrin degradation products

Question 43

When is antifibrinolytic therapy given to someone in DIC?

Answer 43

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

Question 44

What are two prothrombic disorders discussed in class

Answer 44

1. Factor V Leiden 2. HIT

Question 45

What is factor V?

Answer 45

Protein for normal clotting. *Activated protein C inactivates factor V thus stopping clot growth*.

Question 46

What is Factor V Leiden?

Answer 46

Genetic mutation. Abnormal version of Factor V that is resistant to the action of activated protein C. Activated protein C cannot stop factor V Leiden thus excessive fibrin.

Question 47

What does Activated Protein C do?

Answer 47

Inactivates factor V when enough fibrin has been made.

Question 48

Who is usually tested for Factor V Leiden?

Answer 48

**Pregnant women.** Especially ones with unexplained late stage abortions

Question 49

Factor V Leiden is associated with an increased risk of developing an episode of _____ (with or without PE).

Answer 49

DVT

Question 50

Because of the high risks of DVT and PE, Factor V Leiden patients should be on ______

Answer 50

Anticoagulants. - Warfarin - LMWH & unfractionated heparin

Question 51

____ is an autoimmune-mediated drug reaction occurring in as many as 5% of patients after exposure to unfractionated heparin or (rare cases) LMWH.

Answer 51

Heparin-induced Thrombocytopenia (HIT)

Question 52

For HIT patients, the thrombocytopenia occurs ____days after initial therapy.

Answer 52

5-14

Question 53

What is the hallmark sign of HIT?

Answer 53

Low Plt count <100,000 *thrombocytopenia*

Question 54

HIT results in ____ activation and potential____?

Answer 54

platelet; thrombosis (arterial and venous)

Question 55

Evidence suggests that HIT is mediated by immune complexes that are composed of what? (3)

Answer 55

1. IgG antibody 2. Platelet Factor 4 (PF4) 3. Heparin

Question 56

Ptients developing HIT during heparin therapy are at a ______% increased risk for thrombosis

Answer 56

30-75% (absolute risk)

Question 57

A diagnosis of HIT should be entertained for any patient experiencing ____ or ______ during or after heparin administration

Answer 57

thrombosis or thrombocytopenia

Question 58

What is heparin replaced with when HIT is diagnosed?

Answer 58

Agratroban or bivalirudin (direct-thrombin inhibitors)

Question 59

What is Fondaparinux & when is it used?

Answer 59

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

Question 60

Typically, PF4/heparin immune complexes clear from the circulation within _____

Answer 60

3 months