Atypical hemolytic uremic syndrome
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| Section editors | |||
|---|---|---|---|
 |
Shruti Chaturvedi, MBBS, MSCI Baltimore, MD |
 |
Benjamin Tillman, MD Nashville, TN |
2 regimens on this page
2 variants on this page
|
All lines of therapy
Eculizumab monotherapy
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Regimen
| Study | Evidence |
|---|---|
| Legendre et al. 2013 (C08-002) | Phase II |
| Legendre et al. 2013 (C08-003) | Phase II |
| Cofiell et al. 2015 | Non-randomized |
Eligible patients enrolled in Cofiell et al. 2015 had platelet counts less than 150 × 109/L, hemoglobin levels less than or equal to the lower limit of normal, LDH levels greater than or equal to 1.5x upper limit of normal, serum creatinine levels greater than or equal to the upper limit of normal, ADAMTS13 activity greater than or equal to 5%, and no positive Shiga toxin-producing Escherichia coli test.
Therapy
- 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
- C08-002/C08-003: Legendre CM, Licht C, Muus P, Greenbaum LA, Babu S, Bedrosian C, Bingham C, Cohen DJ, Delmas Y, Douglas K, Eitner F, Feldkamp T, Fouque D, Furman RR, Gaber O, Herthelius M, Hourmant M, Karpman D, Lebranchu Y, Mariat C, Menne J, Moulin B, Nürnberger J, Ogawa M, Remuzzi G, Richard T, Sberro-Soussan R, Severino B, Sheerin NS, Trivelli A, Zimmerhackl LB, Goodship T, Loirat C. Terminal complement inhibitor eculizumab in atypical hemolytic-uremic syndrome. N Engl J Med. 2013 Jun 6;368(23):2169-81. link to original article PubMed
- 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 link to PMC article contains verified protocol PubMed