Table 3.

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

^* 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