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Combining an antiviral with rituximab in EBV-related haemophagocytic lymphohistiocytosis led to rapid viral clearance; and a comprehensive review
  1. Christos Stefanou,
  2. Christiana Tzortzi,
  3. Fotini Georgiou,
  4. Chrystalla Timiliotou
  1. Limassol General Hospital, Limassol, Cyprus
  1. Correspondence to Dr Christos Stefanou, stefanouchristos{at}


Epstein-Barr virus (EBV)-related haemophagocytic lymphohistiocytosis (EBVr-HLH) has a better prognosis when the virus is rapidly cleared, but the best antiviral approach is controversial. We present a patient to whom the therapeutic standard rituximab was co-administered with valacyclovir and an HLH-specific treatment with favourable viral and clinical responses. We conducted an extensive literature review and contacted several world reference centres and experts to inquire about their approaches and experience. We conclude that antivirals are infrequently used for EBVr-HLH, despite their laboratory-proven and likely clinical beneficial effect on some EBV-related diseases. However, the role of antivirals remains obscure. Concerns about their lack of efficacy are based on observational data and reports of the cellular tropism of EBV. Therefore, the adjunct use of antivirals may be considered when myelotoxicity is not the primary concern, and related outcomes should be systematically recorded to produce higher quality evidence.

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  • Contributors CS contributed to idea, data collection, review, contacting experts, manuscript. CTz contributed to data collection and critical review. FG contributed to review and organising data. CTi contributed to critical review and supervision.

  • Competing interests None declared.

  • Patient consent Obtained.

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