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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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Cites
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Host-directed immunotherapy to fight infectious diseases
Langelier, M. J., & Vinh, D. C. (2022) · Current Opinion in Pediatrics · doi:10.1097/mop.0000000000001176 · Cited by 9
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“As B-lymphocytes might also play a role in EBVtriggered HPS (35), the use of Rituximab as add-on therapy has been investigated, showing the ability to reduce ferritin levels and EBV titers (36).”
Inflamed—HLH, MAS, or something else?
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“In the correct clinical setting and among other laboratory findings, hemophagocytosis can greatly aid in diagnosing HLH. Prior to the initiation of the HLH-1994 and later HLH2004 treatment protocols, the mortality of untreated HLH was 95% with a median survival of less than 2 months, making early recognition and promp…”
BackgroundMethodInfluentialEtoposide Therapy of Cytokine Storm Syndromes
Henter, J., & Greenwood, T. V. B. (2024) · Advances in experimental medicine and biology · doi:10.1007/978-3-031-59815-9_35 · Cited by 6
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Macrophage Activation Syndrome
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A Decade of Lymphoma-Associated Hemophagocytic Lymphohistiocytosis: Does the Outcome Improve?
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“32 For patients with refractory or relapsed EBV-HLH, the programmed death protein-1 antibody nivolumab led to complete responses in five of seven patients according to a recent clinical trial.”
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“The effectiveness of EBV-loaded B–cell depletion with rituximab in EBV associated HLH has been demonstrated (Chellapandian et al., 2013).”
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Adult-Onset Still's Disease and Macrophage-Activating Syndrome Progressing to Lymphoma
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Host-directed immunotherapy for viral infections
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Infectious Mononucleosis and Other Epstein-Barr Virus–Associated Diseases
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“For example rituximab, an anti-CD20 depleting monoclonal antibody, is often added for EBVtriggered HLH to reduce the virus reservoir in B cells [13].”
BackgroundFirst prospective clinical trial in adult HLH
Rosée, P. G. L. (2015) · Blood · doi:10.1182/blood-2015-09-666503 · Cited by 4
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Hemophagocytic lymphohistiocytosis in a patient with glioblastoma: a case report
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“Rituximab, a monoclonal antibody against CD20, has been shown to be effective in reducing symptoms, inflammation and EBV viral load in patients with EBV-associated HLH [18] .”
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Classification, Clinical Manifestations, and Diagnostics of HLH
Schmid, J. P., Volkmer, B., & Ehl, S. R. (2017) · Springer eBooks · doi:10.1007/978-3-319-59632-7_9 · Cited by 3
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“In 2013, a retrospective study of 42 patients with EBV-associated HLH showed that rituximab in combination with conventional HLHdirected therapies improved symptoms, decreased viral load, diminished inflammation, and reduced transaminitis.(12) In our case, there was a 7-day delay in administering etoposide as her symp…”
BackgroundB‑cell lymphoma‑associated hemophagocytic lymphohistiocytosis: A case report
Ojo, A. S., Asemota, J., Ojukwu, S. G., Rajeh, H., Bot, A., Smith, C., Laziuk, K., & Saleh, M. (2022) · Oncology Letters · doi:10.3892/ol.2022.13365 · Cited by 3
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“No. of cytopenias (Hgb < 9.2 g/l, Leukocyte ≤ 5 x109/l and/or platelet ≤ 110 x 109/l) 0 (1 lineage), 24 (2 lineage), or 34 (3 lineages) Ferritin (mg/l) 0 (<2,000), 35 (2,000–6,000), or 50 (>6,000) Triglyceride (mmol/l) 0 (<1.5), 44 (1.5–4), or 64 (>4) Fibrinogen (g/l) 0 (>2.5) or 30 (≤2.5) Aspartate aminotransferase (…”
BackgroundInfluentialHemophagocytic Lymphohistiocytosis
Lachmann, G., & Brunkhorst, F. M. (2023) · Lessons from the ICU · doi:10.1007/978-3-031-17572-5_4 · Cited by 3
Not So BenignHemophagocytic Lymphohistiocytosis: A Rare Yet Clinically Significant Syndrome
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Hemophagocytic Lymphohistiocytosis in the Context of Hematological Malignancies and Solid Tumors
Zoref‐Lorenz, A., Lehmberg, K., & Jordan, M. B. (2024) · Advances in experimental medicine and biology · doi:10.1007/978-3-031-59815-9_29 · Cited by 3
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A mild, self-resolving case of Epstein-Barr virus-induced hemophagocytic lymphohistiocytosis
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“Many agents have been used in HLH including rituximab, rATG, and alemtuzumab (Mahlaoui et al. 2007; Strout et al. 2010; Chellapandian et al. 2013; Marsh et al. 2013 b ).”
BackgroundMethodHemophagocytic syndrome and Kaposi sarcoma after liver transplantation
Voskanyan, S. E., Евгеньевич, С. В., Maltseva, A. P., Рудаков, В. С., Svetlakova, D. S., Davtyan, A., Simavonyan, Z. K., Gallyamova, А. R., Probatova, N. A., Gorodetskiy, V. R., et al. (2018) · Transplantologiya The Russian Journal of Transplantation · doi:10.23873/2074-0506-2018-10-2-126-141 · Cited by 3
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The X-Linked Lymphoproliferative Syndromes
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“In contrast, EBV-associated HLH in Western populations more commonly involves B cells and is often managed with rituximab (21).”
Hemophagocytic lymphohistiocytosis in patients with inflammatory bowel diseases: a systematic review
Lan, T., Luo, Q., Guo, X., & Jiang, X. (2025) · Frontiers in Immunology · doi:10.3389/fimmu.2025.1575297 · Cited by 2
Lymphoma associated hemophagocytic syndrome: it's going viral
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Hemophagocytic lymphohistiocytosis: Recent progress in the pathogenesis, diagnosis and treatment
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Genetic diseases predisposing to HLH
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“EBVassociated HLH is commonly seen in Asia, and early chemotherapy with the HLH-94 protocol improves the outcome (14, 26, 27).”
BackgroundCytokine Storm Syndrome Associated with Systemic Juvenile Idiopathic Arthritis
Vastert, S. J., Canny, S., Canna, S., Schneider, R., & Mellins, E. (2024) · Advances in experimental medicine and biology · doi:10.1007/978-3-031-59815-9_23 · Cited by 2
Dosanjh, H. S., Dosanjh, P., Ipalawatte, H., & Mehdi, Z. (2025) · Cureus · doi:10.7759/cureus.87066 · Cited by 2
“The initiation of rituximab therapy, typically indicated to improve symptoms, viral load, and inflammation associated with HLH, was held due to the patient’s stable clinical presentation [3].”
CNS Involvement in HLH (CNS-HLH)
Horne, A., & Beutel, K. (2017) · Springer eBooks · doi:10.1007/978-3-319-59632-7_10 · Cited by 2
Macrophage Activation Syndrome
Iweala, O. I., & Wu, E. Y. (2018) · Rare rheumatic diseases · doi:10.1007/978-3-319-99139-9_1 · Cited by 2
Cytokine Storm Syndrome Associated with Systemic Juvenile Idiopathic Arthritis
Schneider, R., Canny, S., & Mellins, E. (2019) · Springer eBooks · doi:10.1007/978-3-030-22094-5_21 · Cited by 2
Dieudonné, Y., Martin, M., Korganow, A., Boutboul, D., & Guffroy, A. (2021) · La Revue de Médecine Interne · doi:10.1016/j.revmed.2021.03.324 · Cited by 2
Xu, H., Xu, X., Cui, G., Fang, J., Chen, W., Xue, M., Jin, R., Chen, H., Zhang, L., & Hu, Y. (2021) · Frontiers in Oncology · doi:10.3389/fonc.2021.681432 · Cited by 2
“reported that combined rituximab with the conventional HLH therapies resulted in a significantly reduction in EBV load and was associated with significant decreasing in ferritin levels (24).”
BackgroundHistory of resaerch on Epstein-Barr virus
Ohga, S. (2014) · Uirusu · doi:10.2222/jsv.64.67 · Cited by 2
Hemophagocytic Lymphohistiocytosis Associated with Malignancies and with Epstein-Barr Virus
Lehmberg, K., Ochi, F., Nichols, K. E., & Ishii, E. (2017) · Springer eBooks · doi:10.1007/978-3-319-59632-7_12 · Cited by 2
Self-citationHajji, M., Barbouch, S., Kaaroud, H., Abdelghani, K. B., Hamida, F. B., Harzallah, A., & Abderrahim, E. (2023) · Clinical Medicine Insights Case Reports · doi:10.1177/11795476231210137 · Cited by 1
Spaner, C., Durkee-Shock, J. R., Weng, A. P., Stubbins, R. J., Gerrie, A. S., Pittaluga, S., Cohen, J. I., & Chen, L. Y. C. (2024) · Frontiers in Hematology · doi:10.3389/frhem.2024.1413794 · Cited by 1
The Cytokine Storm of Multicentric Castleman Disease
Fajgenbaum, D. C. (2024) · Advances in experimental medicine and biology · doi:10.1007/978-3-031-59815-9_31 · Cited by 1
Sakamoto, K., Yanagisawa, R., Ono, R., Doi, T., Negishi, T., Kada, A., Saito, A. M., Kanegane, H., Morimoto, A., Shioda, Y., et al. (2025) · Pediatric Blood & Cancer · doi:10.1002/pbc.32166 · Cited by 1
“Based on previous reports demonstrating the efficacy of rituximab for EBV-HLH [16], we planned rit-uximab administration for the HR group.”
Kanu, V. S. V. R., Ilamurugan, G. P., Wasnik, P. N., Das, P., & Satarkar, R. (2025) · The Journal of Clinical and Scientific Research · doi:10.4103/jcsr.jcsr_5_25 · Cited by 1
Ghonge, A., Samperio, V. M., Hamoud, M., & Dasanu, C. A. (2026) · Expert Opinion on Biological Therapy · doi:10.1080/14712598.2026.2693759 · Cited by 1
“In a retrospective study of 42 patients, rituximab therapy was associated with significant reductions in EBV-viral load and ferritin levels [33].”
A young male with more than lupus
Rudinskaya, A., Shao, M., & Lahita, R. G. (2016) · Lupus · doi:10.1177/0961203316651744 · Cited by 1
Hemophagocytic Lymphohistiocytosis: Diagnosis and Management Challenges
Henry, M. M., & Arceci, R. J. (2016) · Springer eBooks · doi:10.1007/978-3-319-30352-9_52 · Cited by 1
Treatment of Newly Diagnosed HLH and Refractory Disease
Henter, J., Marsh, R. A., & Greenwood, T. V. B. (2017) · Springer eBooks · doi:10.1007/978-3-319-59632-7_14 · Cited by 1
Etoposide Therapy of Cytokine Storm Syndromes
Henter, J., & Greenwood, T. V. B. (2019) · Springer eBooks · doi:10.1007/978-3-030-22094-5_30 · Cited by 1
Х-linked lymphoproliferative syndrome. FEDERAL CLINICAL RECOMMENDATIONS, 2020
Balashov, D., Roppelt, A., Rumjantsev, A. G., & Shcherbina, A. U. (2019) · Russian Journal of Allergy · doi:10.36691/raj.2020.16.4.008 · Cited by 1
[Hemophagocytic lymphohistiocytosis: advances on etiologies, diagnosis and treatment].
Wang, D., & Qiu, H. (2017) · PubMed Central · doi:10.3760/cma.j.issn.0253-2727.2017.03.020 · Cited by 1
X-Linked Lymphoproliferative Disease in Latvia: A Report of Two Clinically Distinct Cases
Nokalna, I., Kreile, M., Butane, D., Kovalova, Z., Daneberga, Z., Miklaševičs, E., Gardovska, D., & Gailīte, L. (2020) · Case Reports in Medicine · doi:10.1155/2020/7108657 · Cited by 1
“For prevention of further sequelae, immune globulin replacement therapy has been used [9], and an alternative for both treatment of disease manifestations and preventative therapy is ablative B-cell therapy with rituximab (anti-CD20), that has shown to control acute primary EBV infection, prevent severe EBV infection,…”
MethodSyndromes of hematophagocytosis in patients of pediatric intensive care units (literature review)
Абрамова, Н. Н., Belozerov, K. E., Кондратьев, Г. В., Аleksаndrovich, Y. S., & Kostik, M. M. (2023) · Messenger of Anesthesiology and Resuscitation · doi:10.24884/2078-5658-2022-20-4-77-88 · Cited by 1
Hemophagocytic Lymphohistiocytosis in a Kidney Transplant Recipient: Case Report
Medina, M. A. P., Prieto, Á. A., Zurutuza, A. P., Amunarriz, C. M., Odriozola, M. J. B., de Barrón, X. C. O., Pérez‐Santaolalla, E., Alonso, O. M., Claveras, I. B., & Tomas, M. T. R. D. (2025) · Transplantation Proceedings · doi:10.1016/j.transproceed.2024.12.012 · Cited by 1
Bouhadana, A., & Steinberg, A. S. (2025) · Clinical Hematology International · doi:10.46989/001c.143659 · Cited by 1
Alhayek, B., Malone, X., MISLAY, J. A., Sentmanat, M. K., Cardew, R. T., & Ramsakal, A. (2025) · Cureus · doi:10.7759/cureus.94918 · Cited by 1
“In North America and most of Europe, the treatment of HLH generally includes dexamethasone and etoposide based on the HLH-94 and HLH-2004 protocols [1,2,16,17].”
Oh, S.-B., & Koh, C.-Y. (2019) · Pediatric Emergency Medicine Journal · doi:10.22470/pemj.2018.00297 · Cited by 1
Hemophagocytic Lymphohistiocytosis in the Context of Hematological Malignancies and Solid Tumors
Lehmberg, K. (2019) · Springer eBooks · doi:10.1007/978-3-030-22094-5_26 · Cited by 1
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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