Table 2.

Revised breakthrough bleed management guidelines.

	Factor (F)VIII	FIX Standard half-life	FIX Extended half-life	APCC	rFVIIa
Recommended single dose	10 IU/kg	20 IU/kg	20 IU/kg	30 U/kg	≤45 μg/kg
Single dose should not exceed	20 IU/kg	30 IU/kg	30 IU/kg	50 U/kg	45 μg/kg
Repeat dosing	Must call clinical study center before second dosea; evaluation and treatment at clinical study center should be considered				Must call clinical study center before third doseb
	Should not repeat in <24 h		Should not repeat in <5-7 d	Should not repeat in <24 h	Should not repeat in <2 h
	For situations requiring higher doses, more frequent administration, or multiple repeated doses, discussion with study medical monitor and clinical advicer is recommended, and AT replacement should be considered.
	Antifibrinolytics should not be used in combination with factor or BPA while on fitusiran.

APCC, activated prothrombin complex concentrate; AT, antithrombin; BPA, bypassing agent; IU, international unit; rFVIIa, recombinant activated factor VII.

Table reproduced with permission from Pipe et al. [60].

^aShould be seen at site within 48 to 72 hours if >2 doses are required.

^bShould be seen at site within 48 to 72 hours if >3 doses are required.