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Epstein-Barr virus associated haemophagocytic lymphohistiocytosis treated with anakinra and rituximab: A case report

McCann, N; Amarnani, R; Shipa, M; Ahmed, S; Thaahira Mohideen, F; Vöö, S; Manson, JJ; (2021) Epstein-Barr virus associated haemophagocytic lymphohistiocytosis treated with anakinra and rituximab: A case report. Clinical Infection in Practice , 9 , Article 100060. 10.1016/j.clinpr.2020.100060. Green open access

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Abstract

BACKGROUND: Haemophagocytic lymphohistiocytosis (HLH) is a severe, life-threatening syndrome characterised by hyperinflammation and macrophage activation. Viral infections such as Epstein-Barr virus (EBV) are a well-recognised trigger of HLH but the treatment of such cases is not well-defined. We present a case of primary EBV driven HLH that was successfully treated with the interleukin-1 inhibitor anakinra in addition to rituximab and high-dose steroids. CASE: A 22-year-old female with no past medical history developed a mononucleosis-like illness lasting five days characterised by fevers, sore throat and neck swelling. Two weeks following this she presented with fevers, night sweats, fatigue and right upper quadrant pain. She was diagnosed with HLH based on high fevers with hyperferritaemia, hypertriglyceridaemia, pancytopaenia, abnormal liver function tests and hepatosplenomegaly. Extensive investigation revealed an EBV viral load of 23,000,000 copies/ml with nil other obvious triggers. A diagnosis of primary-driven EBV HLH was made. She was treated with the interleukin-1 inhibitor anakinra, methylprednisolone and IVIG and a single dose of rituximab. Following the commencement of treatment, the patient made a dramatic improvement. Her EBV viral load reduced to 660 within nine days and her blood counts and liver function returned to normal. She was discharged from hospital on day sixteen. She continued the anakinra for 5 weeks at a weaning dose and completed a 12-week weaning dose of steroids. She has returned to her studies and has no lasting complications from her illness. DISCUSSION: This case highlights the potential of primary EBV infection to cause fulminant HLH. The prompt diagnosis and treatment of HLH using anakinra and rituximab in addition to conventional HLH treatment was safe, and associated with a dramatic clinical improvement. The use of anakinra has been documented in other cases of HLH but none, to our knowledge, of primary EBV-driven HLH with no underlying haematological or rheumatological condition.

Type: Article
Title: Epstein-Barr virus associated haemophagocytic lymphohistiocytosis treated with anakinra and rituximab: A case report
Open access status: An open access version is available from UCL Discovery
DOI: 10.1016/j.clinpr.2020.100060
Publisher version: https://doi.org/10.1016/j.clinpr.2020.100060
Language: English
Additional information: © 2020 The Authors. Published by Elsevier Ltd on behalf of British Infection Association. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Keywords: Epstein-Barr virus, Haemophagocytic lymphohistiocytosis, Anakinra, Rituximab, HLH, EBV
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine > Inflammation
URI: https://discovery.ucl.ac.uk/id/eprint/10130547
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