DOI: 10.12890/2015_000180

Severe Case of Thrombotic Microangiopathy with a Delayed Diagnosis of Atypical Haemolytic Uraemic Syndrome Successfully Managed with Eculizumab

Authors

  • Duaa Aresmouk Nephrocare Hagen GmbH

duaa.aresmouk@nephrocare.com

Keywords

Chronic kidney disease, eculizumab, hemolytic uremic syndrome

Abstract

Objectives: To describe the diagnosis and treatment of a severely ill patient presenting with thrombotic microangiopathy (TMA) of unknown cause.

Case presentation: An adult female presented to intensive care with abdominal pain and haemorrhagic shock, requiring reanimation.

Results: Features of TMA were present, but initial plasma exchange was ineffective. Treatment with the anti-C5 antibody, eculizumab, improved laboratory parameters and organ function, albeit slowly. Eculizumab remains effective and well tolerated after 30 months of treatment.

Conclusion: This case demonstrates the complexities and importance of early identification of atypical haemolytic uraemic syndrome in patients presenting with TMA.

View the entire article

Downloads

References

  1. Campistol JM, Arias M, Ariceta G, Blasco M, Espinosa M, Grinyó JM et al. An update for atypical haemolytic uraemic syndrome: diagnosis and treatment. A consensus document. Nefrologia 2013;33:27–45.
  2. Menne J, Nitschke M, Stingele R, Abu-Tair M, Beneke J, Bramstedt J et al. Validation of treatment strategies for enterohaemorrhagic Escherichia coli O104:H4 induced haemolytic uraemic syndrome: case-control study. BMJ 2012;345:e4565.
  3. Zuber J, Fakhouri F, Roumenina LT, Loirat C, Frémeaux-Bacchi V; French Study Group for aHUS/C3G. Use of eculizumab for atypical haemolytic uraemic syndrome and C3 glomerulopathies. Nat Rev Nephrol 2012;8:643–657.
  4. Legendre CM, Licht C, Muus P, Greenbaum LA, Babu S, Bedrosian C et al. Terminal complement inhibitor eculizumab in atypical hemolytic-uremic syndrome. N Engl J Med 2013;368:2169– 2181.
  5. Povey H, Vundru R, Junglee N, Jibani M. Renal recovery with eculizumab in atypical hemolytic uremic syndrome following prolonged dialysis. Clin Nephrol 2013;82:326–331.
  6. Duran CE, Blasco M, Maduell F, Campistol JM. Rescue therapy with eculizumab in a transplant recipient with atypical haemolytic-uraemic syndrome. Clin Kidney J 2012;5:28–30.

Published

2015-03-25

How to Cite

1.
Aresmouk D. Severe Case of Thrombotic Microangiopathy with a Delayed Diagnosis of Atypical Haemolytic Uraemic Syndrome Successfully Managed with Eculizumab. EJCRIM [Internet]. 2015 Mar. 25 [cited 2026 Nov. 3];2(2). Available from: https://ejcrim.com/index.php/ejcrim/article/view/180