Early eculizumab use in atypical haemolytic uraemic syndrome in a Jehovah's Witness refusing blood products

May Al-Shaghana, Andrew Bentall, Mark D. Jesky, William Lester, Graham Lipkin

Research output: Contribution to journalArticlepeer-review

Abstract

Thrombotic microangiopathy (TMA) is characterized by microscopic angiopathic haemolytic anaemia, thrombocytopenia and organ injury. Supportive therapies include the use of blood products. Recently the terminal complement inhibitor eculizumab has been approved in atypical haemolytic uraemic syndrome (aHUS) in some countries. We report the case of a 23-year-old female Jehovah's Witness presenting with vaginal haemorrhage from thrombocytopaenia, severe anaemia (nadir Hb 28 g/L) and anuric acute kidney injury with TMA secondary to aHUS. Despite a life threatening illness, the patient declined the use of blood components and plasma exchange. Eculizumab was administered early with subsequent improvement and resolution of haemolysis, return to baseline renal function whilst avoiding use of blood products. This case demonstrates the effective use of eculizumab for life saving therapy in a patient refusing blood products. It highlights the importance of accessibility for high cost therapies, but the disparity in access between healthcare systems.

Original languageEnglish (US)
Article numberomx025
Pages (from-to)99-103
Number of pages5
JournalOxford Medical Case Reports
Volume2017
Issue number6
DOIs
StatePublished - Jun 1 2017

ASJC Scopus subject areas

  • Parasitology
  • Microbiology
  • Infectious Diseases

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