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Treatment of Epstein Barr virus‐induced haemophagocytic lymphohistiocytosis with rituximab‐containing chemo‐immunotherapeutic regimens
Open accessAbstract
Summary: Haemophagocytic lymphohistiocytosis ( HLH ) is a life threatening complication of E pstein– B arr virus ( EBV ) infection. The anti‐ CD 20 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/m 2 . 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 .
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“As with EBV-associated HLH,71 rituximab is highly effective in patients with HHV-8–associated multicentric Castleman’s disease, since B-cell depletion removes the primary reservoir for HHV-8.72”
From Cytokine storm (2020) · cites this paper as [71]
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Self-citationAnti-CD20 Antibody Therapy for B-Cell Lymphomas
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“These data support the incorporation of rituximab into future prospective clinical trials for patients with EBV-HLH.”
ResultInfluentialEpstein-Barr virus-associated T-/natural killer cell lymphoproliferative diseases
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BackgroundTreatment advances in posttransplant lymphoproliferative disease
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“4 EBV, a DNA (deoxyribonucleic acid) virus and member of the Herpesviridae family has been the most consistently reported virus associated with HLH. [36] [37] [38] [39] [40] [41] [42] [43] [44] [45] [46] [47] [48] [49] [50] [51] [52] The majority of EBV-associated HLH cases have been reported in Asia, with limited inf…”
Metadata
- Authors
- Chellapandian, D., Das, R., Zelley, K., Wiener, S. J., Zhao, H., Teachey, D. T., Nichols, K. E., & EBV‐HLH Rituximab Study Group
- Affiliations
- Department of Pediatrics Einstein Medical Center Philadelphia PA USA
- Children's Hospital of Philadelphia (US)
- Corresponding author
- Nichols, Kim E.
- Venue
- British Journal of Haematology
- Publisher
- Wiley
- Published
- 2013-05-21
- Volume
- 162
- Issue
- 3
- Pages
- 376-382
- ISSN
- 0007-1048 (ISSN-L)
- 1365-2141
- PMC
- PMC3776423
- OpenAlex
- W2029782045
- Cited by
- 251
- References
- 36
- Citations by year
- 2026: 16 · 2025: 20 · 2024: 22 · 2023: 18 · 2022: 20 · 2021: 25 · 2020: 23 · 2019: 19 · 2018: 19 · 2017: 19 · 2016: 19 · 2015: 14 · 2014: 15 · 2013: 2
- FWCI
- 11.96
- Type
- journal article
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Locations
- British Journal of Haematology (publishedVersion)
- PubMed (publishedVersion)
- PubMed Central (acceptedVersion)
- Funders
- National Heart, Lung, and Blood Institute (R01HL089745)
- National Cancer Institute (K12CA001727)
- Topics
- Health Sciences › Medicine › Hematology › Autoimmune and Inflammatory Disorders Research
- Health Sciences › Medicine › Oncology › Viral-associated cancers and disorders
- Health Sciences › Medicine › Epidemiology › Cytomegalovirus and herpesvirus research
- Keywords
- Epstein-Barr virus, hemophagocytic lymphohistiocytosis, rituximab, anti-CD20 monoclonal antibody, B-cell depletion, EBV viral load, serum ferritin, etoposide, cyclosporine, corticosteroid therapy, chemoimmunotherapy, B-cell lymphoproliferative disorders
- MeSH terms
- Rituximab, Adolescent, Adult, Aged, Antiviral Agents, Child, Child, Preschool, Drug Administration Schedule, Drug Evaluation, Drug Therapy, Combination, Herpesvirus 4, Human, Female, Humans, Immunologic Factors, Male, Middle Aged, Prognosis, Retrospective Studies, Survival Analysis, Treatment Outcome, Viral Load, Epstein-Barr Virus Infections, Lymphohistiocytosis, Hemophagocytic, Young Adult, Antibodies, Monoclonal, Murine-Derived
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