Coagulopathies

Question 1

What’s the most common inherited bleeding disorder?

Answer 1

Von Willebrand disease

Question 2

How is vWD inherited? What are the types?

Answer 2

Autosomal dominant.
Type 1 (75%): decreased vWF
Type 2 (20%): dysfunctional vWF
Type 3 (5%): absence of circulating vWF

Question 3

How does vWF present? How do you treat it?

Answer 3

Bleeding after surgery or dental procedures.
BNleeding: desmopressin and/or CPP w factor 8/vWF
Surgery: Maintain ristocetin cofactor activity/factor 8 activity at 100IU/dL. Postop maintain >50 I U/dL for 5-10 days

Question 4

What’s pathophys/inheritance of hemophilia A and B? How are they treated?

Answer 4

A: X-linked factor 8 deficiency. Recombinant factor 8/9. Desmopressin if mild
B: X-linked factor 9 deficiency. Factor 8/9

Question 5

What’s most common hypercoagulable disorder? What’s pathophys?

Answer 5

Factor V Leiden. Factor V mutation that makes it resistant to inactivation by protein C. Should be anticoagulated regardless of Hx of VTE