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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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BackgroundHow to Treat Involvement of the Central Nervous System in Hemophagocytic Lymphohistiocytosis?
Horne, A., Wickström, R., Jordan, M. B., Yeh, E. A., Naqvi, A., Henter, J., & Janka, G. (2017) · Current Treatment Options in Neurology · doi:10.1007/s11940-017-0439-4 · Cited by 131
Reactions Related to CAR-T Cell Therapy
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BackgroundSelf-citationMacrophage Activation Syndrome
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Severe Epstein–Barr virus infection in primary immunodeficiency and the normal host
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“Rituximab also plays an important role in EBV-driven lymphoproliferative disorder.(56) Further novel therapies including biological agents are on the horizon but evidence so far is scarce.”
BackgroundHemophagocytic lymphohistiocytosis in adults
Bhatt, N. S., Oshrine, B. R., & Talano, J. (2018) · Leukemia & lymphoma/Leukemia and lymphoma · doi:10.1080/10428194.2018.1482543 · Cited by 53
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BackgroundLai, W., Wang, Y., Wang, J., Wu, L., Jin, Z., & Wang, Z. (2018) · Hematology · doi:10.1080/10245332.2018.1491093 · Cited by 51
“monoclonal antibody [23,24], and DEP/L-DEP [25,26].”
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“Rituximab improves overall clinical outcomes and is an important part of EBV clearance in the context of EBV-triggered sHLH or EBV-driven malignancies [21, 68].”
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Ruffner, M. A., Sullivan, K. E., & Henrickson, S. E. (2017) · Frontiers in Immunology · doi:10.3389/fimmu.2017.00665 · Cited by 47
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BackgroundHemophagocytic Lymphohistiocytosis
Kaçar, A. G., & Celkan, T. T. (2022) · Balkan Medical Journal · doi:10.4274/balkanmedj.galenos.2022.2022-4-83 · Cited by 40
Complete spectrum of adverse events associated with chimeric antigen receptor (CAR)-T cell therapies
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“For example, IVIG and rituximab are used for sHLH/ MAS triggered by EBV or EBV-driven malignancies [21, 116, 117].”
BackgroundMacrophage Activation Syndrome
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“66 69 IL-18 blockade is not commercially available but can be considered on a compassionate-use basis (ClinicalTrials.gov identi fi er: NCT04641442, testing the bispeci fi c IL-1 β /IL-18 monoclonal antibody MAS825 in patients with autoin fl ammation due to mutations in NLRC4 or XIAP ).”
Diagnosis and Management of Adult Malignancy-Associated Hemophagocytic Lymphohistiocytosis
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“Importantly, adjunctive therapies during induction should also be considered, such as rituximab for concomitant active EBV infection [60] or intravenous immunoglobulin to treat hypogammaglobulinemia.”
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Bojan, A. S., Pârvu, A. V., Zsoldos, I.-A., Torok, T., & Fărcaș, A. D. (2021) · Experimental and Therapeutic Medicine · doi:10.3892/etm.2021.10336 · Cited by 39
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“Apart from that, Rituximab's introduction is a CD-20 antibody that depletes B cell function, proved to be beneficial as adjuvant treatment [32] .”
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“Some research showed that rituximab-containing regimens appeared well-tolerated and improved clinical status in 43% of patients [27]; however, our center did not find significant improvement compared with a nonrituximab regimen in our small series of EBV-HLH (unpublished observations) [19].”
ResultImmune response to viscerotropic Leishmania: a comprehensive review
Lodi, L., Voarino, M., Stocco, S., Ricci, S., Azzari, C., Galli, L., & Chiappini, E. (2024) · Frontiers in Immunology · doi:10.3389/fimmu.2024.1402539 · Cited by 33
A case of VEXAS syndrome associated with EBV-associated hemophagocytic lymphohistiocytosis
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Cytokine Storm and Sepsis-Induced Multiple Organ Dysfunction Syndrome
Carcillo, J. A., & Shakoory, B. (2024) · Advances in experimental medicine and biology · doi:10.1007/978-3-031-59815-9_30 · Cited by 28
Hemophagocytic lymphohistiocytosis: an update for nephrologists
Filippone, E. J., & Farber, J. L. (2016) · International Urology and Nephrology · doi:10.1007/s11255-016-1294-z · Cited by 27
Shamriz, O., Kumar, D., Shim, J., Briones, M. A., Quarmyne, M., Chonat, S., Lucas, L., Edington, H. J., White, M. H., Mahajan, A., et al. (2021) · Journal of Clinical Immunology · doi:10.1007/s10875-021-01073-9 · Cited by 26
Diagnosis, Treatment, and Management of Hemophagocytic Lymphohistiocytosis in the Critical Care Unit
Hines, M., Bhatt, N. S., & Talano, J. (2019) · Springer eBooks · doi:10.1007/978-3-030-01322-6_9 · Cited by 25
Macrophage Activation Syndrome in Children: Update on Diagnosis and Treatment
Lee, J. Y., Bae, K. S., Rhim, J. W., Lee, S. Y., Jeong, D. C., & Kang, J. H. (2024) · Children · doi:10.3390/children11070755 · Cited by 23
Hemophagocytic Lymphohistiocytosis and Infections
Chesshyre, E. L. D., Ramanan, A. V., & Roderick, M. R. (2018) · The Pediatric Infectious Disease Journal · doi:10.1097/inf.0000000000002248 · Cited by 23
Síndromes hemofagocíticos: la importancia del diagnóstico y tratamiento precoces
Astigarraga, I., González‐Granado, L. I., Allende, L. M., & Alsina, L. (2018) · Anales de Pediatría · doi:10.1016/j.anpedi.2018.05.003 · Cited by 22
Bonnan, M., Ferrari, S., Bertandeau, E., Demasles, S., Krim, E., Miquel, M., & Barroso, B. T. (2014) · Current Drug Targets · doi:10.2174/1389450115666141029234644 · Cited by 21
“The still unsolved problem of Epstein-Barr virus (EBV) persistence in meningeal B-cells [101] should no longer be a problem since CSF clearance from EBV was obtained when intrathecal rituximab was used to treat EBV-induced CNS lymphoma [50, 53] or EBV-induced macrophage activation syndromes [102].”
BackgroundHaemophagocytic syndromes: the importance of early diagnosis and treatment
Astigarraga, I., González‐Granado, L. I., Allende, L. M., & Alsina, L. (2018) · Anales de Pediatría (English Edition) · doi:10.1016/j.anpede.2018.05.002 · Cited by 21
Early Use of Blood Purification in Severe Epstein-Barr Virus–Associated Hemophagocytic Syndrome
Huang, P. Y., Huang, C., Xu, H., Lü, J., Tian, R., Wang, Z., & Chen, Y. (2020) · PEDIATRICS · doi:10.1542/peds.2019-3197 · Cited by 20
Haemophagocytic lymphohistiocytosis: an elusive syndrome
Thomas, W., Veer, M. B. V., & Besser, M. W. (2016) · Clinical Medicine · doi:10.7861/clinmedicine.16-5-432 · Cited by 20
Dilemmas in diagnosis and management of hemophagocytic lymphohistiocytosis in children
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Mostaza-Fernández, J. L., Laso, J. G., Ule, D. C., & Morales, J. M. (2014) · Revista Clínica Española (English Edition) · doi:10.1016/j.rceng.2014.03.004 · Cited by 20
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“Rituximab can be effective for EBV infection [28], but, due to our patient’s rapid improvement with the initial therapy, we did not feel the need to treat him with this agent at that time.”
BackgroundHemophagocytic Lymphohistiocytosis in Patients With Primary Immunodeficiency
Çetinkaya, P. G., Cağdaş, D. N., Gümrük, F., & Tezcan, İ. (2020) · Journal of Pediatric Hematology/Oncology · doi:10.1097/mph.0000000000001803 · Cited by 19
Hämophagozytische Lymphohistiozytose
Lachmann, G., Rosée, P. G. L., Schenk, T., Brunkhorst, F. M., & Spies, C. (2016) · Der Anaesthesist · doi:10.1007/s00101-016-0216-x · Cited by 19
“Die Therapie mit Biologika richtet sich nach der Triggererkrankung: Positive Kasuistiken gibt es für Rituximab bei EBV-getriggerter HLH, um die Replikation des EB-Virus in B-Zellen zu terminieren [66].”
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Macrophage Activation Syndrome in Viral Sepsis
Papageorgiou, D., Gogos, C. A., & Akinosoglou, K. (2024) · Viruses · doi:10.3390/v16071004 · Cited by 18
“As demonstrated in one study, when combined with conventional HLH therapy, rituximab improves symptoms, reduces viral load, and diminishes inflammation [138].”
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Kawamura, Y., Miura, H., Matsumoto, Y., Uchida, H., Kudo, K., Hata, T., Ito, Y., Kimura, H., & Yoshikawa, T. (2016) · BMC Pediatrics · doi:10.1186/s12887-016-0718-3 · Cited by 17
“In a previous report, only 43 % of HLH patients showed improvements following the administration of B-cell depleting agents; however, an initial course of a rituximab-containing regimen may rapidly reduce EBV-DNA load [13].”
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Do COVID-19 Infections Result in a Different Form of Secondary Hemophagocytic Lymphohistiocytosis
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Meyer, L. K., Lee, J. C., Rocco, J. M., & Nichols, K. E. (2025) · Haematologica · doi:10.3324/haematol.2024.286477 · Cited by 14
“103 While IFN g blockade theoretically holds promise in this setting, concerns remain regarding whether suppressing IFN g might impair viral clearance, increasing the risk of persistent or recrudescent infection, though these concerns have been somewhat allayed by a demonstrated improvement in HLH…”
Self-citationAcquired B-cell deficiency secondary to B-cell-depleting therapies
Blincoe, A., Labrosse, R., & Abraham, R. S. (2022) · Journal of Immunological Methods · doi:10.1016/j.jim.2022.113385 · Cited by 12
Wang, W., Yuan, X., Yu, L., & Pei, F. (2024) · Medicine · doi:10.1097/md.0000000000039880 · Cited by 12
“[31] In a specific case, the patient tested negative for EBV DNA after receiving targeted therapy with emapalumab monoclonal antibody, corroborating the research findings.”
ResultAlternative Therapy for Epstein-Barr Virus Related Hemophagocytic Lymphohistiocytosis
Asad, O., Salam, A., Mannem, S. K., Ninan, M. J., Markowitz, A. B., & Jana, B. (2015) · Case Reports in Oncological Medicine · doi:10.1155/2015/508387 · Cited by 12
“These studies demonstrated that rituximab-containing regimens significantly reduce EBV load and signs of inflammation [6, 7].”
Background
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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