Eculizumab

OTHER ADULT INDICATIONS (atypical haemolytic uraemic syndrome [aHUS], refractory generalised myasthenia gravis [gMG] and neuromyelitis optica spectrum disorder [NMOSD], adults 18 years and over): initial phase 900 mg intravenously every week for the first 4 weeks; maintenance phase 1,200 mg at week 5, then 1,200 mg every 14 (plus or minus 2) days. REFRACTORY gMG: clinical response is usually achieved by 12 weeks — consider discontinuing in a patient with no evidence of therapeutic benefit by 12 weeks. aHUS: treatment is recommended to continue for the patient's lifetime unless discontinuation is clinically indicated; monitor for signs and symptoms of thrombotic microangiopathy. PAEDIATRIC (PNH, aHUS or refractory gMG): patients weighing 40 kg or more use the adult regimen. Below 40 kg the regimen is by weight band — 30 to under 40 kg: 600 mg weekly for the first 2 weeks, then 900 mg at week 3 and 900 mg every 2 weeks; 20 to under 30 kg: 600 mg weekly for the first 2 weeks, then 600 mg at week 3 and 600 mg every 2 weeks; 10 to under 20 kg: 600 mg single dose at week 1, then 300 mg at week 2 and 300 mg every 2 weeks; 5 to under 10 kg: 300 mg single dose at week 1, then 300 mg at week 2 and 300 mg every 3 weeks. Paediatric infusions are given over 1-4 hours. Not studied in PNH or refractory gMG patients weighing under 40 kg (the aHUS weight-based regimen is used); safety and efficacy not established in children under 6 years with refractory gMG or under 18 years with NMOSD. SUPPLEMENTAL DOSING WITH PLASMA INTERVENTIONS: plasmapheresis or plasma exchange — if the most recent dose was 300 mg give 300 mg per session within 60 minutes after each session, and if the most recent dose was 600 mg or more give 600 mg per session; fresh frozen plasma infusion — if the most recent dose was 300 mg or more give 300 mg per infusion, 60 minutes before each infusion. SUPPLEMENTAL DOSING WITH IVIg: if the most recent dose was 900 mg or more give 600 mg per IVIg cycle as soon as possible after the cycle; if 600 mg or less give 300 mg per IVIg cycle. ELDERLY: may be administered to patients aged 65 and over with no special precautions, though experience is limited. HEPATIC: safety and efficacy not studied in hepatic impairment. MENINGOCOCCAL COVER: all patients must be vaccinated against Neisseria meningitidis at least 2 weeks before starting, or receive prophylactic antibiotics until 2 weeks after vaccination. HOME INFUSION may be considered for patients who have tolerated clinic infusions well, performed by a qualified healthcare professional. Patients should be monitored after infusion (monitoring duration truncated in the fetched extract).