Atypical hemolytic-uremic syndrome (aHUS)
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All lines of treatment
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|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
- Patients were vaccinated against Neisseria meningitidis or received "appropriate antibiotics" if vaccination occurred within 14 days of the first dose
- 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