How should eculizumab be administered in the treatment of pediatric atypical hemolytic uremic syndrome (aHUS)?

Updated: Nov 12, 2018
  • Author: Robert S Gillespie, MD, MPH; Chief Editor: Craig B Langman, MD  more...
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Answer

Eculizumab therapy should be started immediately upon diagnosis of aHUS. Treatment is given weekly initially and then tapered to every 2 weeks (except for patients weighing < 10 kg, who receive it every 3 wk).

Eculizumab may be used in place of plasma exchange. If patients are receiving concomitant plasma exchange, supplemental eculizumab dosing should be given after plasma exchange.

Inhibition of complement activation increases susceptibility to infection, especially with encapsulated organisms. Patients should receive vaccine for Neisseria meningitidis immediately prior to eculizumab treatment if they have not been immunized or if their immunization status is unknown. Patients should receive antibiotic prophylaxis for N meningitidis for at least 2 weeks following vaccination to allow time for development of immunity.

The optimal duration of eculizumab therapy is unknown and may be life-long. No clear data or guidelines exist to determine criteria for discontinuance. Such decisions must be made on an individual basis and with the understanding that discontinuation is associated with a significant risk of recurrence of thrombotic microangiopathy. In a series of 10 patients with aHUS who discontinued eculizumab therapy, 3 patients relapsed within 6 weeks. [31] If treatment is discontinued, patients should be monitored carefully for signs of relapse.


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