Blood Disorders (Exam II) Marcus's Cards

Question 1

What are the S/S of vWF disorder?

Answer 1

Easy bruising
epistaxis
menorrhagia

Question 2

Von Willabrand Factor Disorder facts

Answer 2

-vWF critical role in platelet adherence/adhesion
-Most common hereditary bleeding disorders

Question 3

Classifications of Inherited vonWillebrand Disease

Answer 3

Type I: Partial quantitative deficiency of vWF
mildest; most common; responds to DDAVP
Type IIA and IIM: Dysfunction of platelet adhesion
May respond to DDAVP
Type IIB: Increased platelet binding affinity
Thrombocytopenia with DDAVP
Type IIN: Decreased F VIII-binding affinity
often confused with hemophilia A
Type III: Severe quantitative deficiency of vWF
Rarest; most severe; usually requires factor concentrates

Question 4

What would lab values be for someone with vWF deficiency?

Answer 4

• Normal PT & aPTT
• Bleeding time is prolonged

Question 5

What are the treatments for vWF deficiency?

Answer 5

• Desmopressin 1st line
• Cryoprecipitate
• Factor VIII (or transfusion of specific factor)

Question 6

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

Answer 6

Stimulates vWF release from endothelial cells (it’s a synthetic analogue of vasopressin)

Question 7

What is the dose for DDAVP?

Answer 7

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

Question 8

What is the Peak & duration of DDAVP?

Answer 8

• Peak: 30mins
• Duration: 6-8hrs

Question 9

What are side effects of DDAVP?

Answer 9

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

Question 10

What is the most major side effect of DDAVP?

Answer 10

Hyponatremia

Question 11

Someone that gets DDAVP needs to be on what?

Answer 11

Fluid restriction 4-6hrs before & after DDAVP

Question 12

What CNS and EKG changes will you see with serum Na of 120 meq/L

Answer 12

CNS: Confusion and restlessness
EKG: maybe widening of QRS

Question 13

What CNS and EKG changes will you see with serum Na of 115 meq/L

Answer 13

CNS: Somnolence and nausea
EKG: elevated ST segments and widened QRS

Question 14

What CNS and EKG changes will you see with serum Na of 110 meq/L

Answer 14

CNS: seizures, coma
EKG: Vtach or Vfib

Question 15

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

Answer 15

Cryoprecipitate

Question 16

1 unit of Cryo raises the ____ level by ___?

Answer 16

Fibrinogen by 50 mg/dL

Question 17

What is a potential risk factor with cryoprecipitate?

Answer 17

• Increased risk of infection (not submitted to viral attenuation)
• Multiple donors

Question 18

How is factor VIII made?

Answer 18

• Pool of plasma from a large number of donors.
• it does undergo viral attenuation

Question 19

What does factor VIII concentrate contain?

Answer 19

Contains Factor VIII and vWF

Question 20

When is Factor VIII given?

Answer 20

Preop or intraop

Question 21

When should DDAVP be given prior to surgery?

Answer 21

60mins before Sx

Question 22

Anesthesia Consideration for vWF disease

Answer 22

• Prior evaluation by a hematologist
• Normalization of the bleeding time and improved levels of F VIII should be confirmed before the surgery in patients
• General anesthesia is preferred
• Patients with coagulopathies undergoing neuroaxial block will have increased risk of developing a hematoma and compression of neurological structures

Question 23

What things to avoid in giving anesthesia to a patient with vWF disease?

Answer 23

• Avoid trauma in anesthesia
• A-lines aren’t recommended
• Laryngeal trauma during intubation may cause a hematoma
• IM medication is avoided

Question 24

What blood product poses an increase risk for infection? Why?

Answer 24

• Cryoprecipitate
• Not sent for viral attenuation

Question 25

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

Answer 25

- Hematoma - Nerve compression

Question 26

How does heparin work?

Answer 26

- Thrombin inhibition: which prevents the conversion of fibrinogen to fibrin - Antithrombin III activation

Question 27

What labs are monitored with heparin? and how can we reverse it?

Answer 27

- PTT &/or ACT - reversible by protamine: a positively charged polypeptide forming a stable complex neutralizing heparin

Question 28

What is the mechanism of action of Coumadin?

Answer 28

Inhibition of vitamin K-dependent factors: II, VII, IX, X

Question 29

What is the onset for Vitamin K administration?

Answer 29

6-8hrs

Question 30

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

Answer 30

- Prothrombin complex concentrates - Factor VIIa - FFP

Question 31

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

Answer 31

**Convert plasminogen to plasmin**, which cleaves fibrin

Question 32

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

Answer 32

**Inhibit conversion of plasminogen to plasmin**

Question 33

Treating patients who are on antiplatelet therapy

Answer 33

- D/C drugs on time - platelet transfusion (if you have to operate emergently) becuase these medicaion irrversibly bind to the platelet

Question 34

What is disseminated intravascular coagulopathy?

Answer 34

- Systemic activation of the coagulation system simultaneously leads to thrombus formation and exhaustion of platelets and coagulation factors - Numerous underlying disorders may precipitate DIC, including trauma, amniotic fluid embolus, malignancy, sepsis (longer onset except for urosepsis), or incompatible blood transfusions

Question 35

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

Answer 35

Treat the underlying cause

Question 36

What will labs show for someone in DIC?

Answer 36

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

Question 37

When is antifibrinolytic therapy given to someone in DIC?

Answer 37

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

Question 38

What should the treatment for DIC include?

Answer 38

Treatment includes blood component transfusions to replete coagulation factors and platelets consumed in the process

Question 39

What is factor V?

Answer 39

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

Question 40

What is Factor V Leiden?

Answer 40

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

Question 41

What does Activated Protein C do?

Answer 41

Inactivates factor V when enough fibrin has been made.

Question 42

Who is usually tested for Factor V Leiden?

Answer 42

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

Question 43

What anticoagulant medications could someone with Factor V Leiden be put on?

Answer 43

- Warfarin - LMWH & unfractionated heparin

Question 44

Anesthesia implications of Factor V Leiden

Answer 44

- Patients will be on anticoagulants becuase they have an increased risk of DVT or PE

Question 45

Pathology of HIT

Answer 45

HIT describes an autoimmune-mediated drug reaction occurring in as many as 5% of patients after exposure to unfractionated heparin or (rare cases) LMWH Thrombocytopenia occurs 5-14 days after initial therapy

Question 46

What is the hallmark sign of HIT?

Answer 46

Plt count <100,000 *thrombocytopenia*

Question 47

HIT results in ____ activation and potential____?

Answer 47

platelet; thrombosis

Question 48

What is heparin replaced with when HIT is diagnosed?

Answer 48

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

Question 49

What is Fondaparinux & when is it used?

Answer 49

- A synthetic Factor Xa inhibitor - used to treat VTE in HIT (off label use) - If possible, avoid future exposure to unfractionated heparin