Recommended schedules of treatment of different clinical situations in patients with recessively inherited coagulation disorders
Factor deficient . | Major surgery . | Minor surgery . | Spontaneous bleeding . | Comments . |
|---|---|---|---|---|
| Fibrinogen | A. Concentrate (20-30 mg/kg) | FFP (15-20 mL/kg) | FFP (15-20 mL/kg) | Prophylaxis with weekly concentrate doses (20-30 mg/kg) if spontaneous bleeding is frequent and severe |
| B. FFP (15-20 mL/kg) | Target: > 50 mg/dL for 2-3 d | Target: > 50 mg/dL until bleeding stops | ||
| C. Cryoprecipitate (1 bag per 10 kg) | ||||
| Target: > 50 mg/dL until healing is complete | ||||
| Prothrombin | A. PCC (20-30 U/kg) | FFP (15-20 mL/kg) | FFP (15-20 mL/kg) | After PCC, FVII, FIX, and FX should not exceed 150% |
| B. FFP (15-20 mL/kg) | Target: > 30% for 2-3 d | Target: > 30% until bleeding stops | ||
| Target: > 30% until healing is complete | ||||
| V | FFP (15-20 mL/kg) | As for major surgery | As for major surgery | No available concentrate |
| Target: > 20% until healing is complete | Target: > 20% for 2-3 d | Target: > 20% until bleeding stops | ||
| VII | A. rFVIIa (15-30 μg/kg at 12-h intervals) | As for major surgery | As for major surgery | Monitor with factor VII activity assays |
| B. Concentrate (30-40 U/kg at 12-h intervals) | Target: > 20% for 2-3 d | Target: > 20% until bleeding stops | ||
| Target: > 20% until healing is complete | ||||
| X | A. PCC (20-30 U/kg) | FFP (15-20 mL/kg) | As for major surgery | After PCC, FII, FVII, and FIX should not exceed 150% |
| B. FFP (15-20 mL/kg) | Target: > 20% for 2-3 d | Target: > 20% until bleeding stops | ||
| Target: > 20% until healing is complete | ||||
| XI | FFP (15-20 mL/kg) | As for major surgery | As for major surgery | Target levels of FXI can usually be achieved also with infusions at alternate days |
| Target: > 20% until healing is complete | Target: > 20% for 2-3 d | Target: > 20% until bleeding stops | ||
| XIII | A. Concentrate (10-20 U/kg) | As for major surgery | As for major surgery | Prophylaxis in all patients. The replacement material can be infused every 20-30 d |
| B. FFP (15-20 mL/kg) | Target: > 5% for 2-3 d | Target: > 5% until bleeding stops | ||
| C. Cryoprecipitate (1 bag per 10 kg) | ||||
| Target: > 5% until healing is complete |
Factor deficient . | Major surgery . | Minor surgery . | Spontaneous bleeding . | Comments . |
|---|---|---|---|---|
| Fibrinogen | A. Concentrate (20-30 mg/kg) | FFP (15-20 mL/kg) | FFP (15-20 mL/kg) | Prophylaxis with weekly concentrate doses (20-30 mg/kg) if spontaneous bleeding is frequent and severe |
| B. FFP (15-20 mL/kg) | Target: > 50 mg/dL for 2-3 d | Target: > 50 mg/dL until bleeding stops | ||
| C. Cryoprecipitate (1 bag per 10 kg) | ||||
| Target: > 50 mg/dL until healing is complete | ||||
| Prothrombin | A. PCC (20-30 U/kg) | FFP (15-20 mL/kg) | FFP (15-20 mL/kg) | After PCC, FVII, FIX, and FX should not exceed 150% |
| B. FFP (15-20 mL/kg) | Target: > 30% for 2-3 d | Target: > 30% until bleeding stops | ||
| Target: > 30% until healing is complete | ||||
| V | FFP (15-20 mL/kg) | As for major surgery | As for major surgery | No available concentrate |
| Target: > 20% until healing is complete | Target: > 20% for 2-3 d | Target: > 20% until bleeding stops | ||
| VII | A. rFVIIa (15-30 μg/kg at 12-h intervals) | As for major surgery | As for major surgery | Monitor with factor VII activity assays |
| B. Concentrate (30-40 U/kg at 12-h intervals) | Target: > 20% for 2-3 d | Target: > 20% until bleeding stops | ||
| Target: > 20% until healing is complete | ||||
| X | A. PCC (20-30 U/kg) | FFP (15-20 mL/kg) | As for major surgery | After PCC, FII, FVII, and FIX should not exceed 150% |
| B. FFP (15-20 mL/kg) | Target: > 20% for 2-3 d | Target: > 20% until bleeding stops | ||
| Target: > 20% until healing is complete | ||||
| XI | FFP (15-20 mL/kg) | As for major surgery | As for major surgery | Target levels of FXI can usually be achieved also with infusions at alternate days |
| Target: > 20% until healing is complete | Target: > 20% for 2-3 d | Target: > 20% until bleeding stops | ||
| XIII | A. Concentrate (10-20 U/kg) | As for major surgery | As for major surgery | Prophylaxis in all patients. The replacement material can be infused every 20-30 d |
| B. FFP (15-20 mL/kg) | Target: > 5% for 2-3 d | Target: > 5% until bleeding stops | ||
| C. Cryoprecipitate (1 bag per 10 kg) | ||||
| Target: > 5% until healing is complete |
Clinical situations and treatment options in order of priority. FFP denotes fresh-frozen plasma, preferably virus-inactivated; PCC, prothrombin complex concentrate; and rFVIIa, recombinant FVIIa.