Table 1.

Definition of factor replacement protocol and prophylaxis regimen choice [6].

Protocol		Prophylaxis Regimen Choice
Continuous prophylaxis		If initiated with the intent of treating for 52 weeks of the year and is accomplished for at least 45 weeks of that year
	Primary prophylaxis	The regular, continuous use of treatment that is started before the second clinically evident large joint bleed (bleeds in ankles, knees, hips, elbows, or shoulders). It is initiated before three years of age in children without documented osteochondral joint disease (determined by a physical examination and/or imaging studies)
	Secondary prophylaxis	Initiated after ≥2 bleeds into large joints but before the onset of joint disease documented by physical examination and imaging studies
	Tertiary prophylaxis	The continuous treatment started after the onset of joint disease, as documented by physical examination and plain radiographs of the affected joints
Intermittent prophylaxis		If the prophylaxis regimen does not exceed more than 45 weeks in a year
“On-demand” therapy		Treatment given at the time of clinically evident bleeding