Atypical hemolytic uremic syndrome

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Shruti Chaturvedi, MBBS, MSCI
Johns Hopkins University
Baltimore, MD

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Benjamin Tillman, MD
Vanderbilt University
Nashville, TN
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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.

Immunosuppressive therapy

  • Eculizumab (Soliris) as follows:
    • Cycle 1: 900 mg IV once per day on days 1, 8, 15, 22
    • Cycle 2 onwards: 1200 mg IV once on day 1

Supportive medications

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

28-day cycle for 1 cycle, then 14-day cycles

References

  1. 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
  2. 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