Eculizumab

Source: UK SPC (eMC) for BEKEMV 300 mg concentrate for solution for infusion, section 4.2 (https://www.medicines.org.uk/emc/product/15378/smpc). The aHUS regimen is quoted above as the renal-relevant indication. SEPARATE INDICATION — PNH IN ADULTS (different doses, do NOT mix with the aHUS regimen): initial phase 600 mg IV weekly for the first 4 weeks; maintenance phase 900 mg for the fifth week, then 900 mg every 14 plus or minus 2 days. SUPERVISION: must be administered by a healthcare professional under the supervision of a physician experienced in the management of patients with haematological and renal disorders. Home infusion may be considered for patients who have tolerated infusions well in the clinic, after evaluation and recommendation by the treating physician, and must be performed by a qualified healthcare professional; there is limited safety data supporting home-based infusions and additional precautions (availability of emergency treatment of infusion reactions or anaphylaxis) are recommended. SUPPLEMENTAL DOSING WITH PLASMA INTERVENTIONS: plasmapheresis or plasma exchange — if the most recent dose was 300 mg, give a supplemental 300 mg per session; if the most recent dose was 600 mg or more, give a supplemental 600 mg per session; timing, within 60 minutes after each session. Fresh frozen plasma infusion — if the most recent dose was 300 mg or more, give a supplemental 300 mg per infusion, 60 minutes prior to each fresh frozen plasma infusion. SUPPLEMENTAL DOSING WITH IVIg: if the most recent dose was 900 mg or more, give a supplemental 600 mg per IVIg cycle as soon as possible after the cycle; if the most recent dose was 600 mg or less, give a supplemental 300 mg per IVIg cycle. TREATMENT MONITORING AND DURATION: aHUS patients should be monitored for signs and symptoms of thrombotic microangiopathy; treatment is recommended to continue for the patient's lifetime unless discontinuation is clinically indicated. INFUSION PRACTICALITIES: patients should be monitored for one hour following infusion; if an adverse event occurs the infusion may be slowed or stopped at the discretion of the physician, but if slowed the total infusion time may not exceed two hours in adults and four hours in paediatric patients under 18 years. Paediatric infusions are given over 1 - 4 hours. It is not necessary to protect the diluted solution from light during administration. PAEDIATRIC (fixed weight-band doses, NOT a per-kg dose, so paedDose is left null): paediatric PNH and aHUS patients weighing 40 kg or more are treated with the adult dosing recommendations. For patients above 2 years of age weighing less than 40 kg — 30 to less than 40 kg: 600 mg weekly for the first 2 weeks, then 900 mg at week 3 and 900 mg every 2 weeks; 20 to less than 30 kg: 600 mg weekly for the first 2 weeks, then 600 mg at week 3 and 600 mg every 2 weeks; 10 to less than 20 kg: 600 mg single dose at week 1, then 300 mg at week 2 and 300 mg every 2 weeks; 5 to less than 10 kg: 300 mg single dose at week 1, then 300 mg at week 2 and 300 mg every 3 weeks. CONTRAINDICATED in babies and young children below 2 years of age. Eculizumab has not been studied in patients with PNH who weigh less than 40 kg; the posology used in paediatric PNH patients below 40 kg is identical to the weight-based aHUS recommendation. Verify all paediatric dosing against a children's formulary. ELDERLY: may be administered to patients aged 65 and over with no special precautions indicated, although experience in this population is limited. HEPATIC IMPAIRMENT: safety and efficacy have not been studied. SOURCE LIMITATION: eMC section 4.5 was NOT captured in this bundle — the interactions listed below come from section 7 of the US label (BKEMV, Amgen, label date 2026-07-02, openFDA/DailyMed) and must be checked against the UK SPC. Sections 4.4 and 4.8 were truncated at the fetch limit.