Severe Case of Thrombotic Microangiopathy with a Delayed Diagnosis of Atypical Haemolytic Uraemic Syndrome Successfully Managed with Eculizumab
Keywords
Chronic kidney disease, eculizumab, hemolytic uremic syndromeAbstract
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.