CEN Case Reports

, Volume 8, Issue 2, pp 139–143 | Cite as

Three months interval therapy of Eculizumab in a patient with atypical hemolytic uremic syndrome with hybrid CFHR1/CFH gene

  • Sami AlobaidiEmail author
  • Ammar AlDabbagh
  • Amany Alamoudi
  • Murad Almowarey
  • Ahmed AKL
Case Report


Pregnancy-associated atypical hemolytic uremic syndrome (P-aHUS) is a rare condition. It is characterized by very high maternal mortality and morbidity. Most cases of P-aHUS (79%) manifest in the postpartum period; this is probably due to the complement’s involvement in aHUS pathogenesis. Eculizumab is approved for aHUS treatment, but its use is limited due to cost, unknown duration of treatment, and vague dose intervals to keep patients in remission. In this case report, we present a 26-year-old female with P-aHUS with hybrid CFHR1/CFH gene. Eculizumab was initiated after 5 weeks of being on hemodialysis and plasmapheresis sessions. Full remission successfully achieved after 6th dose of Eculizumab, within 13 weeks of onset of aHUS. Due to financial issues and inability to financially cover the cost, Eculizumab was set in hold. Within 6 months, she suffered recurrence of the disease and Eculizumab was re-instated. After re-inducing full remission, the patient was switched to Eculizumab every 3 months instead of the recommended manufacture dose interval of every 2 weeks. We followed this patient for 3 years and she continued to be in remission based on clinical and laboratory data. In conclusion, achievement of successful and maintenance of remission of P-aHUS in this patient who had limited access to Eculizumab raise the attention of the efficacy of Eculizumab at longer time intervals. However, it is time to consider conducting a long-term study to learn about the safety and efficacy of this approach, which may have a major financial advantage for patients.


Eculizumab aHUS Complement dysregulation nephropathy Pregnancy-aHUS 



We would like to thank Prof. Fadi Fakhouri, MD, PhD, Centre Hospitalier, Universitaire de Nantes, Nantes (CHU Nantes), FRANCE for reviewing the manuscript and for his helpful comments and suggestions.

Compliance with ethical standards

Conflict of interest

The authors did not report any potential conflicts of interest.

Ethical standards

The present study was performed in accordance with the ethical standards of the institutional research committee and with the Helsinki declaration.

Informed consent

Written consent was obtained from the patient discussed and documentation is available for review upon request.


  1. 1.
    Franchini M. Atypical hemolytic uremic syndrome: from diagnosis to treatment. ClinChem Lab Med. 2015;53(11):1679–88.Google Scholar
  2. 2.
    Kistler AD. [Atypical hemolytic uremic syndrome (aHUS): new insights into pathogenesis leading to novel therapeutic approaches]. Praxis (Bern 1994). 2016;105(7):389–96.CrossRefGoogle Scholar
  3. 3.
    Sawai T, Nangaku M, Ashida A, et al. Diagnostic criteria for atypical hemolytic uremic syndrome proposed by the Joint Committee of the Japanese Society of Nephrology and the Japan Pediatric Society. Pediatr Int. 2014;56(1):1–5.CrossRefGoogle Scholar
  4. 4.
    Bajracharya P, Jain A, Baracco R, Mattoo TK, Kapur G. Atypical hemolytic uremic syndrome: a clinical conundrum. Pediatr Nephrol. 2016;31(10):1615–24.CrossRefGoogle Scholar
  5. 5.
    Eyler SJ, Meyer NC, Zhang Y, Xiao X, Nester CM, Smith RJ. A novel hybrid CFHR1/CFH gene causes atypical hemolytic uremic syndrome. Pediatr Nephrol. 2013;28(11):2221–5.CrossRefGoogle Scholar
  6. 6.
    De sousaamorim E, Blasco M, Quintana L, Sole M, De cordoba SR, Campistol JM. Eculizumab in pregnancy-associated atypical hemolytic uremic syndrome: insights for optimizing management. J Nephrol. 2015;28(5):641–5.CrossRefGoogle Scholar
  7. 7.
    Fakhouri F, Roumenina L, Provot F, et al. Pregnancy-associated hemolytic uremic syndrome revisited in the era of complement gene mutations. J Am Soc Nephrol. 2010;21(5):859–67.CrossRefGoogle Scholar
  8. 8.
    Legendre CM, Licht C, Muus P, et al. Terminal complement inhibitor eculizumab in atypical hemolytic-uremic syndrome. N Engl J Med. 2013;368(23):2169–81.CrossRefGoogle Scholar
  9. 9.
    Fakhouri F, Hourmant M, Campistol JM, et al. Terminal complement inhibitor Eculizumab in adult patients with atypical hemolytic uremic syndrome: a single-arm, open-label trial. Am J Kidney Dis. 2016;68(1):84–93.CrossRefGoogle Scholar
  10. 10.
    Walle JV, Delmas Y, Ardissino G, Wang J, Kincaid JF, Haller H. Improved renal recovery in patients with atypical hemolytic uremic syndrome following rapid initiation of eculizumab treatment. J Nephrol. 2016;30:127–34.CrossRefGoogle Scholar
  11. 11.
    Fakhouri F, Fila M, Provôt F, et al. Pathogenic variants in complement genes and risk of atypical hemolytic uremic syndrome relapse after Eculizumab discontinuation. Clin J Am SocNephrol. 2017;12(1):50–9.CrossRefGoogle Scholar
  12. 12.
    Saland JM, Ruggenenti P, Remuzzi G. Liver-kidney transplantation to cure atypical hemolytic uremic syndrome. J Am SocNephrol. 2009;20(5):940–9.Google Scholar
  13. 13.
    Jiang H, Fan MN, Yang M, et al. Association among complement factor H autoantibodies, deletions of CFHR, and the risk of atypical hemolytic uremic syndrome. Int J Environ Res Public Health. 2016;13(12):1209.CrossRefGoogle Scholar
  14. 14.
    Sarris I, Gandhi S, Koumis A, et al. Pregnancy outcome and safety of breast-feeding in two patients with paroxysmal nocturnal haemoglobinuria (PNH) treated with eculizumab. Arch Dis Child Fetal Neonatal Ed. 2012;97:A119. (Abstract PP.43).CrossRefGoogle Scholar
  15. 15.
    Aydin S, Audisio E, Iovino G, et al. Breakthrough hemolysis controlled by eculizumab escalation during pregnancy in paroxysmal noctural hemoglobinuria (PNH): a single case report. Haematologica. 2017;102(Suppl 3):58 (Abstract).Google Scholar

Copyright information

© Japanese Society of Nephrology 2019

Authors and Affiliations

  1. 1.Department of MedicineDr.Soliman Fakeeh HospitalJeddahSaudi Arabia
  2. 2.Department of MedicineUniversity of JeddahJeddahSaudi Arabia
  3. 3.Nephrology Department, Urology and Nephrology CenterMansoura UniversityMansouraEgypt

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