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CASE REPORT
Crohn's disease complicated by Epstein-Barr virus-driven haemophagocytic lymphohistiocytosis successfully treated with rituximab
  1. Grace Thompson1,
  2. Dominic Pepperell2,
  3. Ian Lawrence3,4,
  4. Benjamin David McGettigan1
  1. 1Department of Immunology, Fiona Stanley Hospital, Murdoch, Western Australia, Australia
  2. 2Department of Haematology, Fiona Stanley Hospital, Murdoch, Western Australia, Australia
  3. 3Harry Perkins Institute of Medical Research Perkins South, Perth, Bahrain
  4. 4Centre for Inflammatory Bowel Diseases, Subiaco, Perth, Western Australia, Australia
  1. Correspondence to Dr Grace Thompson, grace.thompson{at}health.wa.gov.au

Summary

We report a case of Epstein-Barr virus (EBV)-driven haemophagocytic lymphohistiocytosis (HLH) in a man with Crohn's disease treated with 6-mercaptopurine and adalimumab therapy who was successfully treated with rituximab therapy alone. This is the first published case in an adult patient with EBV-driven HLH in the setting of thiopurine use and inflammatory bowel disease to be successfully treated with rituximab therapy alone. Here, we will discuss putative immunological mechanisms which may contribute to this potentially life-threatening complication.

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Footnotes

  • Contributors GT is responsible for planning of the manuscript and primarily responsible for writing the manuscript. BMG is responsible for planning, editing and finalising the manuscript. DP and IL contributed equally to editing and finalising the manuscript.

  • Competing interests None declared.

  • Patient consent Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.