6/16 - Coagulation Disorders Treatment

Question 1

What is in FFP and when do we use it? What’s the problem with it?

Answer 1

Contains all procoagulants and anticoagulants – Each 1cc of FFP has 1 unit of factor activity

Volume about 250cc

Used when diagnosis not known, when multiple factors needed, or concentrated factor product not available

Problem is volume overload

Question 2

Cryoprecipitate

Answer 2

FFP thawed at 4 degrees C

Concentrated for: Fibrinogen, F 8, 13, and vWF

Less volume than FFP – About 15-30cc

Same risks as FFP except less for volume overload

Question 3

How do we treat hemophilia A?

Answer 3

Plasma Derived Factor Concentrates

Example: Monoclate P (Pasturized, monoclonal Ab purified)

Question 4

How much of a factor 8 concentrate should we give?

Answer 4

Dose for 8 = Wt (kg) X Desired (% increase) X 0.5

Example: Increase FV 8 level to 30% in severe hemophilia A patient weighing 50 kg

50 kg X 30 X 0.5 = 750 units F 8

Question 5

Recombinate vs. Advate

Answer 5

Recombinate has Albumin stabilizer and is the oldest

Advate is newer and has no Albumin, is stabilized instead by sucrose and has no risk for human pathogen transmission

Both are Factor 8 recombinants

Question 6

How do we treat Hemophilia B?

Answer 6

Recombinant Factor 9

Question 7

How do we dose for Factor 9?

Answer 7

Dose of IX = Wt (kg) X Desired (% increase) X 1.2

Example: Raise FIX level to 80% in Hemophilia B patient weighing 70 kg

Dose= 70 X 80 X 1.2 = 6720 units

Question 8

What is Alprolix?

Answer 8

Long acting, Fc fusion protein product

It is a recombinant IgG Complexed Factor 9

Question 9

Desmopressin

Answer 9

Synthetic analogue of vasopressin (antidiuretic hormone)

Factor VIII and VWF increases by 4-6X via release from endothelial stores

Question 10

Novoseven

Answer 10

Direct activation of extrinsic pathway - Direct thrombin burst