Musculoskeletal problems of people with hemophilia aged > 65 years

Table 1 Factor replacement schedule for people with hemophilia A or B aged > 65 years without inhibitors based on World Federation of Hemophilia guidelines

Type of surgery	Hemophilia A		Hemophilia B
	Aim peak levels (%)	Time span	Aim peak levels (%)	Time span
Minor orthopedic surgery	50-80	Day of surgery	50-80	Day of surgery
	30-80	Days 1-5	30-80	Days 1-5
Major orthopedic surgery	80-100	Day of surgery	60-80	Day of surgery
	60-80	Days 1-3	40-60	Days 1-3
	40-60	Days 4-6	30-50	Days 4-6
	30-50	Days 7-14	20-40	Days 7-14

Dosing must be individualized according to factor level monitoring, thrombotic risk and center protocols.

Table 2 Dosing regimens of bypassing agents in people with hemophilia with inhibitors aged > 65 years

Drug	Dosing for minor orthopedic surgery	Dosing for major orthopedic surgery	Comments
Recombinant factor VII activated	90 µg/kg/dose immediately before surgery and every 2 hours for 2 days and then every 2-6 hours until healed	90 µg/kg/dose immediately before surgery and every 2 hours for 2 days. Then every 2-3 hours for 5 days, then every 4 hours until days 7-10, then every 6 hours until days 14-21	Preferred drug for individuals with hemophilia B and high-titer inhibitors per WFH guidelines since aPCCs contains factor IX and may cause or worsen an allergic reaction
			For individuals with factor IX deficiency, low dose (15-30 µg/kg) in combination with tranexamic acid has been shown to be effective for major surgery in limited studies and can be considered for those at risk of thrombosis
aPCCs	50-100 U/kg immediately prior to surgery and then every 6-12 hours	50-100 U/kg immediately prior to surgery, then every 6-12 hours	Maximum: 100 units/kg/dose; 200 units/kg/day)
			Less preferred for hemophilia B individuals with high-titer inhibitors per WFH guidelines

Dosing must be individualized according to thrombotic risk and center protocols. aPCCs: Activated prothrombin complex concentrate; WFH: World Federation of Hemophilia.