Treatment of E pstein B arr 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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Rituximab is highly effective in patients with HHV-8-associated multicentric Castleman's disease because B-cell depletion removes the primary reservoir for HHV-8.
“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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Imashuku, S., Morimoto, A., & Ishii, E. (2021) · Acta Paediatrica · doi:10.1111/apa.15973 · Cited by 98 · see citing works
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Clinical features and correct diagnosis of macrophage activation syndrome
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“More recently, there have been several reports of the benefit of rituximab (B cell depleting antibody directed to CD20) for treating EBV-induced MAS, particularly in the setting of immunodeficiency states prone to EBV infection [61].”
BackgroundEvolution of Our Understanding of XIAP Deficiency
Mudde, A. C. A., Booth, C., & Marsh, R. A. (2021) · Frontiers in Pediatrics · doi:10.3389/fped.2021.660520 · Cited by 89 · see citing works
Ruxolitinib for secondary hemophagocytic lymphohistiocytosis
Sin, J. H., & Zangardi, M. L. (2017) · Hematology/Oncology and Stem Cell Therapy · doi:10.1016/j.hemonc.2017.07.002 · Cited by 86 · see citing works
Hemophagocytic Lymphohistiocytosis in Adults
Campo, M. J., & Berliner, N. (2015) · Hematology/Oncology Clinics of North America · doi:10.1016/j.hoc.2015.06.009 · Cited by 83 · see citing works
Jessen, B., Kögl, T., Sepulveda, F. E., Basile, G. D. S., Aichele, P., & Ehl, S. R. (2013) · Frontiers in Immunology · doi:10.3389/fimmu.2013.00448 · Cited by 77 · see citing works
“Further research in HLH patients will be needed to address this important issue, since treatment of a triggering infection can be an important component of successful therapy for HLH (37, 74, 75).”
BackgroundHighways to hell: Mechanism-based management of cytokine storm syndromes
Canna, S., & Cron, R. Q. (2020) · Journal of Allergy and Clinical Immunology · doi:10.1016/j.jaci.2020.09.016 · Cited by 73 · see citing works
“However, rituximab works best in treating HLH associated with XLP.(73)”
Hereditary and Acquired Hemophagocytic Lymphohistiocytosis
Zhang, L., Zhou, J., & Sokol, L. (2014) · Cancer Control · doi:10.1177/107327481402100406 · Cited by 72 · see citing works
“Although intravenous immunoglobulin has been recommended for the treatment of reactivated EBV infection, patients with EBV-associated B-lymphoproliferative disorders taking rituximab have also been found to have improved outcomes.(105) The efficacy of a rituximab-containing regimen was investigated in 42 patients with…”
BackgroundInfluentialWang, J., Wang, Y., Wu, L., Zhang, J., Lai, W., & Wang, Z. (2016) · Journal of Hematology & Oncology · doi:10.1186/s13045-016-0317-7 · Cited by 61 · see citing works
“[20] retrospectively analyzed the clinical information of 42 patients with EBV-HLH who received a rituximab monoclonal antibody treatment regimen.”
BackgroundMethodHow i treat primary haemophagocytic lymphohistiocytosis
Marsh, R. A., & Haddad, É. (2018) · British Journal of Haematology · doi:10.1111/bjh.15274 · Cited by 61 · see citing works
Shakoory, B., Geerlinks, A. V., Wilejto, M., Kernan, K. F., Hines, M., Romano, M., Piskin, D., Ravelli, A., Sinha, R. R., Aletaha, D., et al. (2023) · Arthritis & Rheumatology · doi:10.1002/art.42636 · Cited by 60 · see citing works
“B cell depletion may be useful in some patients with EBV-HLH (103,104).”
BackgroundSelf-citationMacrophage Activation Syndrome
Sen, E. S., Clarke, S. L. N., & Ramanan, A. V. (2015) · The Indian Journal of Pediatrics · doi:10.1007/s12098-015-1877-1 · Cited by 59 · see citing works
Severe Epstein–Barr virus infection in primary immunodeficiency and the normal host
Worth, A. J. J., Houldcroft, C. J., & Booth, C. (2016) · British Journal of Haematology · doi:10.1111/bjh.14339 · Cited by 58 · see citing works
“Supportive care, particularly to prevent and treat infection is critical, and in EBV-HLH, removal of the infec- tious trigger with multiple courses of Rituximab is essential (Chellapandian et al, 2013).”
BackgroundYanagisawa, R., Nakazawa, Y., Matsuda, K., Yasumi, T., Kanegane, H., Ohga, S., Morimoto, A., Hashii, Y., Imaizumi, M., Okamoto, Y., et al. (2018) · International Journal of Hematology · doi:10.1007/s12185-018-02572-z · Cited by 57 · see citing works
Diagnosing haemophagocytic syndrome
Sen, E. S., Steward, C. G., & Ramanan, A. V. (2016) · Archives of Disease in Childhood · doi:10.1136/archdischild-2016-310772 · Cited by 55 · see citing works
Clinical features and outcomes in secondary adult hemophagocytic lymphohistiocytosis
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Haemophagocytic lymphohistiocytosis in adult critical care
Bauchmüller, K., Manson, J. J., Tattersall, R. S., Brown, M., McNamara, C., Singer, M., & Brett, S. J. (2020) · Journal of the Intensive Care Society · doi:10.1177/1751143719893865 · Cited by 54 · see citing works
“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 · see citing works
“Additionally, Rituximab (CD20-specific antibody) can effectively result in B-lymphocyte depletion, eliminating a reservoir of EBV, and has shown benefit as adjunctive treatment [65], but cannot be used as monotherapy because of ability of EBV to affect T/NK cells in HLH.”
BackgroundLai, W., Wang, Y., Wang, J., Wu, L., Jin, Z., & Wang, Z. (2018) · Hematology · doi:10.1080/10245332.2018.1491093 · Cited by 51 · see citing works
“monoclonal antibody [23,24], and DEP/L-DEP [25,26].”
ResultCattaneo, C. M., Oberti, M., Skert, C., Passi, A., Farina, M., Re, A., Tozzi, P., Borlenghi, E., & Rossi, G. (2016) · Hematological Oncology · doi:10.1002/hon.2314 · Cited by 50 · see citing works
Song, Y., Wang, Y., & Wang, Z. (2019) · British Journal of Haematology · doi:10.1111/bjh.15988 · Cited by 50 · see citing works
Sandler, R. D., Carter, S. J., Kaur, H., Francis, S., Tattersall, R. S., & Snowden, J. A. (2019) · Bone Marrow Transplantation · doi:10.1038/s41409-019-0637-7 · Cited by 50 · see citing works
“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].”
BackgroundRecurrent and Sustained Viral Infections in Primary Immunodeficiencies
Ruffner, M. A., Sullivan, K. E., & Henrickson, S. E. (2017) · Frontiers in Immunology · doi:10.3389/fimmu.2017.00665 · Cited by 47 · see citing works
“MMWR Morb Mortal Wkly Rep (1994) 43(28):505–9.”
BackgroundHemophagocytic Lymphohistiocytosis
Kaçar, A. G., & Celkan, T. T. (2022) · Balkan Medical Journal · doi:10.4274/balkanmedj.galenos.2022.2022-4-83 · Cited by 40 · see citing works
Complete spectrum of adverse events associated with chimeric antigen receptor (CAR)-T cell therapies
Yang, C., Nguyen, J., & Yen, Y. (2023) · Journal of Biomedical Science · doi:10.1186/s12929-023-00982-8 · Cited by 40 · see citing works
“For example, IVIG and rituximab are used for sHLH/ MAS triggered by EBV or EBV-driven malignancies [21, 116, 117].”
BackgroundMacrophage Activation Syndrome
Nigrovic, P. A. (2024) · Arthritis & Rheumatology · doi:10.1002/art.43052 · Cited by 40 · see citing works
“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
Lee, J. C., & Logan, A. C. (2023) · Cancers · doi:10.3390/cancers15061839 · Cited by 39 · see citing works
“Importantly, adjunctive therapies during induction should also be considered, such as rituximab for concomitant active EBV infection [60] or intravenous immunoglobulin to treat hypogammaglobulinemia.”
BackgroundMacrophage activation syndrome: A diagnostic challenge (Review)
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 · see citing works
Sztajnbok, F., Fonseca, A. R., Campos, L. R., Lino, K., Rodrigues, M. C. F., Silva, R. M., de Almeida, R. G., Perazzio, S. F., & Carvalho, M. D. F. F. (2024) · Revista Brasileira de Reumatologia · doi:10.1186/s42358-024-00370-2 · Cited by 38 · see citing works
Meng, G., Wang, J., Wang, Y., Wei, N., & Wang, Z. (2021) · International Immunopharmacology · doi:10.1016/j.intimp.2021.107606 · Cited by 38 · see citing works
Hemophagocytic Lymphohistiocytosis and Infection: A Literature Review
Koumadoraki, E., Madouros, N., Sharif, S., Saleem, A., Jarvis, S., & Khan, S. (2022) · Cureus · doi:10.7759/cureus.22411 · Cited by 38 · see citing works
“Apart from that, Rituximab's introduction is a CD-20 antibody that depletes B cell function, proved to be beneficial as adjuvant treatment [32] .”
BackgroundDiaz, L., Jauzelon, B., Dilies, A. C., Souder, C. L., Faillie, J., Maria, A. T. J., & Palassin, P. (2023) · Journal of Clinical Medicine · doi:10.3390/jcm12051985 · Cited by 35 · see citing works
Jin, Z., Wang, Y., Wang, J., Wu, L., Pei, R., Lai, W., & Wang, Z. (2017) · Hematology · doi:10.1080/10245332.2017.1385191 · Cited by 34 · see citing works
“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 · see citing works
A case of VEXAS syndrome associated with EBV-associated hemophagocytic lymphohistiocytosis
Kao, R. L., Jacobsen, A. A., Billington, C. J., Yohe, S. L., Beckman, A. K., Vercellotti, G. M., & Pearson, D. R. (2021) · Blood Cells Molecules and Diseases · doi:10.1016/j.bcmd.2021.102636 · Cited by 33 · see citing works
Nußhag, C., Morath, C. P., Zeier, M. G., Weigand, M. A., Merle, U., & Brenner, T. (2017) · Medicine · doi:10.1097/md.0000000000009283 · Cited by 32 · see citing works
The Multifaceted Immunology of Cytokine Storm Syndrome
Lee, P. Y., & Cron, R. Q. (2023) · The Journal of Immunology · doi:10.4049/jimmunol.2200808 · Cited by 32 · see citing works
Wang, J., Wang, Y., Wu, L., & Wang, Z. (2014) · Annals of Hematology · doi:10.1007/s00277-014-2276-9 · Cited by 30 · see citing works
Mostaza-Fernández, J. L., Laso, J. G., Ule, D. C., & Morales, J. M. (2014) · Revista Clínica Española · doi:10.1016/j.rce.2014.03.009 · Cited by 29 · see citing works
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 · see citing works
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 · see citing works
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 · see citing works
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 · see citing works
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 · see citing works
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 · see citing works
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 · see citing works
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 · see citing works
“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 · see citing works
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 · see citing works
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 · see citing works
Dilemmas in diagnosis and management of hemophagocytic lymphohistiocytosis in children
Xu, X., & Tang, Y. (2019) · World Journal of Pediatrics · doi:10.1007/s12519-019-00299-3 · Cited by 20 · see citing works
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 · see citing works
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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 · see citing works
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 · see citing works
“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].”
Berger, J. R., & Kakara, M. (2022) · Multiple Sclerosis and Related Disorders · doi:10.1016/j.msard.2022.103678 · Cited by 18 · see citing works
Macrophage Activation Syndrome in Viral Sepsis
Papageorgiou, D., Gogos, C. A., & Akinosoglou, K. (2024) · Viruses · doi:10.3390/v16071004 · Cited by 18 · see citing works
“As demonstrated in one study, when combined with conventional HLH therapy, rituximab improves symptoms, reduces viral load, and diminishes inflammation [138].”
BackgroundChesner, J. H., Schiano, T. D., Fiel, M. I., & Crismale, J. F. (2021) · Clinical Transplantation · doi:10.1111/ctr.14392 · Cited by 18 · see citing works
He, H., Lei, F., Huang, L., Wang, X., Yang, Y., Chen, L., Peng, Y. F., Liang, Y., Tan, H., Wu, X., et al. (2023) · Biomedicine & Pharmacotherapy · doi:10.1016/j.biopha.2023.114797 · Cited by 17 · see citing works
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“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].”
BackgroundChi, Y., Liu, R., Zhou, Z., Shi, X., Ding, Y., & Li, J. (2021) · Pediatric Rheumatology · doi:10.1186/s12969-021-00534-0 · Cited by 16 · see citing works
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 13 · see citing works
“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 · see citing works
Wang, W., Yuan, X., Yu, L., & Pei, F. (2024) · Medicine · doi:10.1097/md.0000000000039880 · Cited by 12 · see citing works
“[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 · see citing works
“These studies demonstrated that rituximab-containing regimens significantly reduce EBV load and signs of inflammation [6, 7].”
Background
- 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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