Table 1.

Route of administration and standard dosing recommendations for approved complement inhibitors in adult patients with PNH

DrugTargetRouteLoading doseMaintenance doseFrequency
Eculizumab C5 Intravenous over 1 h 600 mg weekly ×4 doses; maintenance dosing starting week 5 900 mg Every 2 wk 
Ravulizumab C5 Intravenous over 1 h ≥40 to <60 kg: 2400 mg
≥60 to <100 kg: 2700 mg
≥100 kg: 3000 mg;
start maintenance dosing 2 wk later 
≥40 to <60 kg: 3000 mg
≥60 to <100 kg: 3300 mg
≥100 kg: 3600 mg 
Every 8 wk 
Pegcetacoplan C3 Subcutaneous infusion over 30-60 min Overlap with C5 inhibitor for 4 wk 1080 mg Twice weekly; every 3 d for LDH >2× ULN 
DrugTargetRouteLoading doseMaintenance doseFrequency
Eculizumab C5 Intravenous over 1 h 600 mg weekly ×4 doses; maintenance dosing starting week 5 900 mg Every 2 wk 
Ravulizumab C5 Intravenous over 1 h ≥40 to <60 kg: 2400 mg
≥60 to <100 kg: 2700 mg
≥100 kg: 3000 mg;
start maintenance dosing 2 wk later 
≥40 to <60 kg: 3000 mg
≥60 to <100 kg: 3300 mg
≥100 kg: 3600 mg 
Every 8 wk 
Pegcetacoplan C3 Subcutaneous infusion over 30-60 min Overlap with C5 inhibitor for 4 wk 1080 mg Twice weekly; every 3 d for LDH >2× ULN 

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