Von willebrand Disease

Question 1

What is the defect in type 1C vWd? How is the diagnosis made?

Answer 1

AKA the vichenza variant, this is due to increased cleavage of vWf. The propeptide level will be elevated.

Question 2

What ratio amount corresponds to type 2A, 2B, and 2M?

Answer 2

A VWF activity:VWF antigen less than 0.6 indicates any of these types of VWD.

Question 3

What ratio corresponds to type 1C?

Answer 3

A pro-peptide to VWF antigen ratio greater than 4.

Question 4

What ratio should make you consider Type 2N?

Answer 4

If you have a factor VIII to VWF antigen ratio less than 0.6. OR VWF antigen to factor VIII is greater than 3.

Question 5

What differentiates type 2M from type 2A and 2B??!

Answer 5

In type 2M the multimeters are normal!!! Please remember this to quickly answer this question!

Question 6

How do you make the diagnosis of platelet type VwD vs type 2B VwD?

Answer 6

The low dose RIPA will be prolonged in patient derived platelets only with platelet type VwD and in Type 2B disease it will only be prolonged with patient derived plasma (where VWF is).

Question 7

What is the treatment of type 2B vs platelet type VWD?

Answer 7

Type 2B-VWF concentrate
Platelet Type-platelet transfusion