Active Ingredient: Eculizumab
Eculizumab is indicated in adults and children for the treatment of paroxysmal nocturnal haemoglobinuria (PNH). Evidence of clinical benefit is demonstrated in patients with haemolysis with clinical symptom(s) indicative of high disease activity, regardless of transfusion history.
For this indication, competent medicine agencies globally authorize below treatments:
Intravenous
300 - 1,200 mg
From 300 To 1,200 mg once every 7 day(s)
Paediatric PNH and aHUS patients with body weight ≥ 40 kg are treated with the adult dosing recommendations, respectively.
In paediatric PNH and aHUS patients with body weight below 40 kg, the eculizumab dosing regimen consists of:
| Patient Body Weight | Initial Phase | Maintenance Phase |
|---|---|---|
| 30 to <40 kg | 600 mg weekly x 2 | 900 mg at week 3; then 900 mg every 2 weeks |
| 20 to <30 kg | 600 mg weekly x 2 | 600 mg at week 3; then 600 mg every 2 weeks |
| 10 to <20 kg | 600 mg weekly x 1 | 300 mg at week 2; then 300 mg every 2 weeks |
| 5 to <10 kg | 300 mg weekly x 1 | 300 mg at week 2; then 300 mg every 3 weeks |
Eculizumab has not been studied in patients with PNH who weigh less than 40kg. The posology of eculizumab for PNH patients less than 40kg weight is based on the posology used for patients with aHUS and who weigh less than 40kg.
Eculizumab has not been studied in paediatric patients with refractory gMG or NMOSD.
For adult aHUS, refractory gMG and NMOSD patients and paediatric aHUS patients supplemental dosing of eculizumab is required in the setting of concomitant PE/PI (plasmapheresis or plasma exchange, or fresh frozen plasma infusion):
| Type of Plasma Intervention | Most Recent eculizumab Dose | Supplemental eculizumab Dose With Each PE/PI Intervention | Timing of Supplemental eculizumab Dose |
|---|---|---|---|
| Plasmapheresis or plasma exchange | 300 mg | 300 mg per each plasmapheresis or plasma exchange session | Within 60 minutes after each plasmapheresis or plasma exchange |
| ≥600 mg | 600 mg per each plasmapheresis or plasma exchange session | ||
| Fresh frozen plasma infusion | ≥300 mg | 300 mg per infusion of fresh frozen plasma | 60 minutes prior to each infusion of fresh frozen plasma |
aHUS patients should be monitored for signs and symptoms of thrombotic microangiopathy (TMA). Eculizumab treatment is recommended to continue for the patient’s lifetime, unless the discontinuation of eculizumab is clinically indicated.
The diluted solution of eculizumab should be administered by intravenous infusion over 25–45 minutes (35 minutes ± 10 minutes) in adults.
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