Coagulation

Question 1

What receptor on the platelets is responsible for binding VWF?

Answer 1

Gp1b-IX

Question 2

What is the role of PAI-1?

Answer 2

It inhibits both t-PA and urokinase

Question 3

What is the role of alpha 2 antiplasmin?

Answer 3

It inhibits plasmin

Question 4

Why is it imperative to avoid the use of FEIBA with Emicizumab (Hemlibra)?

Answer 4

To avoid the formation of thrombosis and TMA.

Question 5

Using Amicar and FEIBA can lead to what and should be avoided?

Answer 5

Thrombosis

Question 6

How does Hemlibra work and what is the molecule?

Answer 6

It is a bi-specific antibody that works by activating factor X by linking factor X and IX together.

Question 7

What type of product in Hemophilia A is associated with a higher risk of inhibitor development? What is the recommendation?

Answer 7

Recombinant has a higher risk over Plasma product. However, there is no recommendation to use one over the other.

Question 8

What is the unique presentation of the development of a factor IX inhibitor? There are two events that can occur.

Answer 8

Anaphylaxis or anaphylactoid reaction esp in patients with null factor IX mutations. Also patients can develop nephrotic syndrome.

Question 9

What is contained in FEIBA and how does it work?

Answer 9

FEIBA contains factors II, VII, IX, and X. It works primarily through factor II and X.

Question 10

How does novo seven and seven fact work? And what type of product is it?

Answer 10

It works by TF independent FX and thrombin generation on the surface of activated platelets. It’s a recombinant product.

Question 11

What are the common pathway factors meaning the factors that will lead to an elevation of the PT and PTT?

Answer 11

Factors1, 2, 5, and 10.

Question 12

What are the factors involved in the intrinsic pathway that lead to elevation of the PTT alone?

Answer 12

Factors 8,9,11, and 12.