Protocols - Hemorio

TABLE 6 – REPLACEMENT THERAPY IN LESS FREQUENT INHERITED COAGULOPATHIES
Deficiency Product Initial Dose Maintenance Dose
Fibrinogen Cryoprecipitated 1,5 U Cryo/10 kg According to plasma levels
Prothrombin Plasma
Prothrombin tablet
80
15 ml/kg
20 IU/Kg
10 ml/kg (daily)
10 IU/kg (daily)
Factor V P.F.C 20 ml/kg 10 ml/kg every 12 h
Factor VII
Factor X
Plasma
Prothrombin tablet
Plasma
Prothrombin tablet
10 ml/kg
10 IU./kg
15 ml/kg
15 IU/kg
5 ml/kg (daily)
10 IU/kg (daily)
10 ml/kg (daily)
10 IU/kg (daily)
Factor XI Plasma 10 ml/kg 5 ml/kg (daily)
Factor XIII
Cryoprecipitated 1U Cryo/ 10kg every 2 or 3 week
CFXIII (not available by MS) 20 IU/Kg Every 28 days
4- PURPURA TREATMENTS DUE TO PLATELET DISORDERS
- Glanzmann’s thrombasthenia
- Bernard-Soulier syndrome
- Purpura by Granule Deficiency
- Purpura with changes to platelet release mechanism - SPD
GENERAL MEASURES
- Ice on site
- Compressive bandages
- Antifibrinolytics
PLATELET CONCENTRATE
Prepare 1U to each 10 kg of weight 1-2 times a day. Prepare the immunophenotyping of patient’s HLA
platelets at the time of diagnosis.
DDAVP
Patients with Glanzmann disease do not respond to DDAVP. Other patients with thrombopathy must
undergo therapeutic test.