Treatment of Epstein Barr virus-induced haemophagocytic lymphohistiocytosis with rituximab-containing chemo-immunotherapeutic regimens

Deepakbabu Chellapandian, Rupali Das, Kristin Zelley, Susan J. Wiener, Huaqing Zhao, David T. Teachey, Kim E. Nichols

Research output: Contribution to journalArticlepeer-review

Abstract

Haemophagocytic lymphohistiocytosis (HLH) is a life threatening complication of Epstein-Barr virus (EBV) infection. The anti-CD20 antibody rituximab depletes B cells, leading to improved outcomes for patients with EBV-associated B-lymphoproliferative disorders. To gather data on the use of rituximab in EBV-HLH, we performed a retrospective investigation involving 42 EBV-HLH patients who had received treatment with rituximab-containing regimens. On average, patients received 3 rituximab infusions (range 1-10) at a median dose of 375 mg/m2. In all patients, rituximab was administered with other HLH-directed medications, including steroids, etoposide and/or ciclosporin. Rituximab-containing regimens appeared well tolerated and improved clinical status in 43% of patients. Examination of laboratory data obtained prior to and within 2-4 weeks after the first rituximab dose revealed significant reductions in EBV load (median load pre-rituximab: 114 200 copies/ml, median post-rituximab: 225 copies/ml, P = 0·0001) and serum ferritin levels (median ferritin pre-rituximab: 4260 μg/l, median post-rituximab: 1149 μg/l, P = 0·001). Thus, when combined with conventional HLH-directed therapies, rituximab improves symptoms, reduces viral load and diminishes inflammation. These data support the incorporation of rituximab into future prospective clinical trials for patients with EBV-HLH.

Original languageEnglish (US)
Pages (from-to)376-382
Number of pages7
JournalBritish journal of haematology
Volume162
Issue number3
DOIs
StatePublished - Aug 2013
Externally publishedYes

Keywords

  • Epstein-Barr virus
  • Haemophagocytic lymphohistiocytosis
  • Macrophage activation syndrome
  • Rituximab
  • X-linked lymphoproliferative disease

ASJC Scopus subject areas

  • Hematology

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