Heme #7: Hemo A/B, VWD, Factor V, C/S, Anti 3

Question 1

Hemophilia A and B are X-linked recessive disorders occurring almost exclusively in ______

Hemophilia A is a deficiency of _____
Hemophilia B is a deficiency of _____

Answer 1

Almost always in males

A: Factor 8 deficiency
B: Factor 9 deficiency

Question 2

Symptoms of Hemophilia A/B

Answer 2

-Hemarthrosis: delayed bleeding or swelling in weight-bearing joints (ankles, knees, elbows) and muscles (forehead hematoma).
-Excessive hemorrhage due to trauma, surgery, or incisional bleeding

Question 3

Diagnostics for Hemophilia A/B
-CBC
-Mixing studies (does it correct)
-What is the most sensitive lab?

Answer 3

-CBC: Prolonged aPTT. Normal platelets, PT, fibrinogen
-PTT corrects with mixing studies
-Low factor VIII/IX (most sensitive)

Question 4

Management of Hemophilia A

Answer 4

Factor VIII infusion (first line therapy)

Desmopressin (DDAVP) increases Factor VIII and VWF from stores. This can be used prior to procedures to prevent bleeding.

Question 5

Management of Hemophilia B

Answer 5

Factor IX infusion

-Desmopressin is NOT useful in Hemophilia B

Question 6

What is the MC type of Hemophilia?

Answer 6

Hemophilia A