Hemostatic management of rare bleeding disorders and precautions in older adult patients
| Bleeding disorder . | Recommended hemostatic treatment for minor surgery . | Recommended hemostatic treatment for major surgery . | Suggested target trough level of deficient factor . |
|---|---|---|---|
| Rare factor deficiencies | |||
| II | FFP (15-20 mL/kg) | FFP (15-20 mL/kg) PCC* (20-30 U/kg) | >30% |
| V | FFP (15-20 mL/kg) | FFP (15-20 mL/kg) | >20% |
| V + VIII | FFP (15-20 mL/kg) | FFP (15-20 mL/kg) | Factor V: >20% FVIII: as in Table 1 |
| VII | rFVIIa (15-30 µg /kg every 4-6 hours) | rFVIIa (15-30 µg/kg every 4-6 hours) PCC* (20-30 mL/kg) | >20% |
| X | Factor X concentrate (10-20 U/kg) FFP 15-20 mL/kg | Factor X concentrate (10-20 U/kg) FFP (15-20 mL/kg) PCC* (20-30 U/kg) | >20% |
| XIII | Plasma-derived factor XIII concentrate (40 U/kg) Recombinant factor XIII concentrate (35 U/kg) Cryoprecipitate (1 bag per 10 kg) | Plasma-derived factor XIII concentrate (40 U/kg) Recombinant factor XIII concentrate (35 U/kg) Cryoprecipitate (1 bag per 10 kg) | 5% |
| Other rare bleeding disorders | |||
| Platelet function defects | Intravenous or oral TXA (500-1000 mg every 6-12 hours) Desmopressin | Platelet transfusions (4-6 units) Bypass agents† | |
| Hypo/dysfibrinogenemia† | Cryoprecipitate (1 bag per 10 kg) Fibrinogen concentrate (20-30 mg/kg) FFP (15-20 mL/kg) | Cryoprecipitate (1 bag per 10 kg) Fibrinogen concentrate (20-30 mg/kg) | >100 mg/dL |
| Disorders of fibrinolysis | Intravenous or oral TXA (500-1000 mg every 6-12 hours) | Intravenous TXA (10-15 mg/kg) every 6-12 hours | |
| Bleeding disorder . | Recommended hemostatic treatment for minor surgery . | Recommended hemostatic treatment for major surgery . | Suggested target trough level of deficient factor . |
|---|---|---|---|
| Rare factor deficiencies | |||
| II | FFP (15-20 mL/kg) | FFP (15-20 mL/kg) PCC* (20-30 U/kg) | >30% |
| V | FFP (15-20 mL/kg) | FFP (15-20 mL/kg) | >20% |
| V + VIII | FFP (15-20 mL/kg) | FFP (15-20 mL/kg) | Factor V: >20% FVIII: as in Table 1 |
| VII | rFVIIa (15-30 µg /kg every 4-6 hours) | rFVIIa (15-30 µg/kg every 4-6 hours) PCC* (20-30 mL/kg) | >20% |
| X | Factor X concentrate (10-20 U/kg) FFP 15-20 mL/kg | Factor X concentrate (10-20 U/kg) FFP (15-20 mL/kg) PCC* (20-30 U/kg) | >20% |
| XIII | Plasma-derived factor XIII concentrate (40 U/kg) Recombinant factor XIII concentrate (35 U/kg) Cryoprecipitate (1 bag per 10 kg) | Plasma-derived factor XIII concentrate (40 U/kg) Recombinant factor XIII concentrate (35 U/kg) Cryoprecipitate (1 bag per 10 kg) | 5% |
| Other rare bleeding disorders | |||
| Platelet function defects | Intravenous or oral TXA (500-1000 mg every 6-12 hours) Desmopressin | Platelet transfusions (4-6 units) Bypass agents† | |
| Hypo/dysfibrinogenemia† | Cryoprecipitate (1 bag per 10 kg) Fibrinogen concentrate (20-30 mg/kg) FFP (15-20 mL/kg) | Cryoprecipitate (1 bag per 10 kg) Fibrinogen concentrate (20-30 mg/kg) | >100 mg/dL |
| Disorders of fibrinolysis | Intravenous or oral TXA (500-1000 mg every 6-12 hours) | Intravenous TXA (10-15 mg/kg) every 6-12 hours | |
After PCC treatment, factors II, VI, IX, and X should not exceed 150%.
Dysfibrinogenemia often presents with a thrombotic phenotype. Hypofibrinogenemia and afibrinogenemia usually present with bleeding, although both venous and arterial thrombosis can occur. Replacement recommendations apply to patients with a bleeding phenotype, and concomitant antithrombotic prophylaxis may be necessary in some, particularly older adults.
Adapted with permission from Mannucci et al.67