10.19.18 Hemorrhagic Disorders

Question 1

What is the most common inherited bleeding disorder?

Answer 1

Von Willebrand Disease

Question 2

What is the pattern of inheritance for VWD?

Answer 2

Autosomal co-dominant

Question 3

What is Type I VWD?

Answer 3

Quantitative disorder- levels about 30% of normal

Question 4

What are the clinical manifestations of VWD?

Answer 4

1. Mucocutaneous bleeding
2. Excessive bleeding after surgery- esp on mucosal surfaces
3. Menorrhagia

NOT joint/muscle bleeding

Question 5

How do you diagnose VWD?

Answer 5

1. Personal history of abnormal bleeding
2. Family history of abnormal bleeding
3. Low levels of VWF (<30$)

Question 6

How do you treat VWD?

Answer 6

1. Desmopressin (DDVAP)

2. Blood products/clotting factors– plasma-derived factor concentrates that contain F8 and vWF

Question 7

What can happen with prolonged use of DDAVP?

Answer 7

Hyponatremia

Question 8

What are the differences in lab testing between Hemophilia A and Type 1 VWD?

Answer 8

PFA100 normal in HA but prolonged in VWD

VWF antigen/activity normal in HA but low in VWD

Question 9

What is used to treat mild hemophilia A (not B)

Answer 9

DDAVP

Question 10

Why can women have low levels of F8 or F9?

Answer 10

Lyonization