Atypical haemolytic uremic syndrome (aHUS)

Active Ingredient: Eculizumab

Indication for Eculizumab

Population group: only children (1 year - 12 years old) , adolescents (12 years - 18 years old)

Eculizumab is indicated in adults and children for the treatment of atypical haemolytic uremic syndrome (aHUS).

For this indication, competent medicine agencies globally authorize below treatments:

300-1200 mg once a week

Route of admnistration


Defined daily dose

300 - 1,200 mg

Dosage regimen

From 300 To 1,200 mg once every 7 day(s)

Detailed description

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 WeightInitial Phase Maintenance Phase
30 to <40 kg600 mg weekly x 2900 mg at week 3; then 900 mg every 2 weeks
20 to <30 kg600 mg weekly x 2600 mg at week 3; then 600 mg every 2 weeks
10 to <20 kg600 mg weekly x 1300 mg at week 2; then 300 mg every 2 weeks
5 to <10 kg300 mg weekly x 1300 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 InterventionMost Recent eculizumab DoseSupplemental eculizumab Dose With Each PE/PI InterventionTiming of Supplemental eculizumab Dose
Plasmapheresis or plasma exchange300 mg300 mg per each plasmapheresis or plasma exchange sessionWithin 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 plasma60 minutes prior to each infusion of fresh frozen plasma

Treatment monitoring

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.

Dosage considerations

The diluted solution of eculizumab should be administered by intravenous infusion over 25–45 minutes (35 minutes ± 10 minutes) in adults.

Active ingredient


Eculizumab is a monoclonal antibody, a terminal complement inhibitor that specifically binds to the complement protein C5 with high affinity, thereby inhibiting its cleavage to C5a and C5b and preventing the generation of the terminal complement complex C5b-9. Eculizumab preserves the early components of complement activation that are essential for opsonization of microorganisms and clearance of immune complexes.

Read more about Eculizumab

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