Atypical hemolytic-uremic syndrome (aHUS)

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1 regimens on this page
1 variants on this page


All lines of treatment

Eculizumab (Soliris)

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Regimen

Study Evidence
Cofiell et al. 2015 Non-randomized

Eligible patients had platelet counts < 150 × 109/L, hemoglobin levels ≤ lower limit of normal, LDH levels ≥ 1.5x upper limit of normal, serum creatinine levels ≥ upper limit of normal, ADAMTS13 activity ≥ 5%, and no positive Shiga toxin-producing Escherichia coli test.

  • Eculizumab (Soliris) 900 mg IV once per week for 4 weeks, then 1200 mg IV once on week 5, then 1200 mg IV every 2 weeks

Supportive medications:

  • Patients were vaccinated against Neisseria meningitidis or received "appropriate antibiotics" if vaccination occurred within 14 days of the first dose

Indefinite course

References

  1. Cofiell R, Kukreja A, Bedard K, Yan Y, Mickle AP, Ogawa M, Bedrosian CL, Faas SJ. Eculizumab reduces complement activation, inflammation, endothelial damage, thrombosis, and renal injury markers in aHUS. Blood. 2015 May 21;125(21):3253-62. Epub 2015 Apr 1. link to original article contains verified protocol PubMed