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Original Article

Treatment Strategies for Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis (EBV-HLH)

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Pages 37-49 | Received 12 Dec 1999, Published online: 01 Jul 2009

Keep up to date with the latest research on this topic with citation updates for this article.

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James W. Verbsky & William J. Grossman. (2006) Hemophagocytic lymphohistiocytosis: Diagnosis, pathophysiology, treatment, and future perspectives. Annals of Medicine 38:1, pages 20-31.
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Hiroshi Gomyo, Manabu Shimoyama, Kentaro Minagawa, Kimikazu Yakushijin, Norinaga Urahama, Atsuo Okamura, Katsuya Yamamoto, Mitsuhiro Ito, Kazuo Chihara & Toshimitsu Matsui. (2003) Effective Anti-viral Therapy for Hemophagocytic Syndrome Associated with B-cell Lymphoma. Leukemia & Lymphoma 44:10, pages 1807-1810.
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Articles from other publishers (32)

Huan Xu, Xia Xu, Guohui Cui, Jun Fang, Wanxin Chen, Mei Xue, Runming Jin, Hongbo Chen, Lu Zhang & Yu Hu. (2021) Secondary Hemophagocytic Lymphohistiocytosis With Epstein–Barr Virus-Associated Transformed Follicular Lymphoma: A Case Report and Literature Review. Frontiers in Oncology 11.
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Chen Zhang, Zhenzhen Liang, Shumei Ma & Xiaodong Liu. (2021) Radiotherapy and Cytokine Storm: Risk and Mechanism. Frontiers in Oncology 11.
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Han-Seung Park, Jung-Hee Lee, Je-Hwan Lee, Eun-Ji Choi, Sun-Hye Ko, Miee Seol, Young-Shin Lee, Young-Ah Kang, Mijin Jeon & Kyoo-Hyung Lee. (2019) Fludarabine/Melphalan 100 mg/m2 Conditioning Therapy Followed by Allogeneic Hematopoietic Cell Transplantation for Adult Patients with Secondary Hemophagocytic Lymphohistiocytosis. Biology of Blood and Marrow Transplantation 25:6, pages 1116-1121.
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Samuel A. Merrill, Rakhi Naik, Michael B. Streiff, Satish Shanbhag, Sophie Lanzkron, Evan M. Braunstein, Alison M. Moliterno & Robert A. Brodsky. (2018) A prospective quality improvement initiative in adult hemophagocytic lymphohistiocytosis to improve testing and a framework to facilitate trigger identification and mitigate hemorrhage from retrospective analysis. Medicine 97:31, pages e11579.
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Jaroslaw P. Maciejewski & Swapna Thota. 2018. Hematology. Hematology 425 444.e5 .
Esraa M. Eloseily & Randy Q. Cron. 2018. The Microbiome in Rheumatic Diseases and Infection. The Microbiome in Rheumatic Diseases and Infection 151 182 .
Solaf Elsayed, Ezzat Elsobky, Azza Tantawy, Eman Ragab, Marine Gil, Nathalie Lambert & Geneviève de Saint Basile. (2016) Wolman disease in patients with familial hemophagocytic lymphohistiocytosis (FHL) negative mutations. Egyptian Journal of Medical Human Genetics 17:3, pages 277-280.
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Jong Jin Seo. (2015) Hematopoietic cell transplantation for hemophagocytic lymphohistiocytosis: recent advances and controversies. Blood Research 50:3, pages 131.
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Valentina Ansuini, Donato Rigante & Susanna Esposito. (2013) Debate around infection-dependent hemophagocytic syndrome in paediatrics. BMC Infectious Diseases 13:1.
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Kazuyuki Matsuda, Yozo Nakazawa, Ryu Yanagisawa, Takayuki Honda, Eiichi Ishii & Kenichi Koike. (2011) Detection of T-cell receptor gene rearrangement in children with Epstein–Barr virus-associated hemophagocytic lymphohistiocytosis using the BIOMED-2 multiplex polymerase chain reaction combined with GeneScan analysis. Clinica Chimica Acta 412:17-18, pages 1554-1558.
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Akane Kunitomi, Hiroshi Kimura, Yoshinori Ito, Kazuyuki Naitoh, Nobuhiko Noda, Hiroatsu Iida & Hiroshi Sao. (2011) Unrelated Bone Marrow Transplantation Induced Long-Term Remission in a Patient with Life-Threatening Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis. Journal of Clinical and Experimental Hematopathology 51:1, pages 57-61.
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Nadine Rouphael Maakaroun, Abeer Moanna, Jesse T Jacob & Helmut Albrecht. (2010) Viral infections associated with haemophagocytic syndrome. Reviews in Medical Virology 20:2, pages 93-105.
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Lam T. My, Le B. Lien, Wen-Chuan Hsieh, Toshihiko Imamura, Tran N. K. Anh, Phan N. L. Anh, Nguyen T. Hung, Fan-Chen Tseng, Chia-Yu Chi, Ngo T. H. Dao, Duong T. M. Le, Le Q. Thinh, Tran T. Tung, Shinsaku Imashuku, Tang C. Thuong & Ih-Jen Su. (2010) Comprehensive analyses and characterization of haemophagocytic lymphohistiocytosis in Vietnamese children. British Journal of Haematology 148:2, pages 301-310.
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Yu-Hsiang Chang, Pei-Jung Lu, Meng-Yao Lu, Jyh-Seng Wang, Chun-Liang Tung & Chen-Fu Shaw. (2009) Sequential Transplants for Respective Relapse of Hodgkin Disease and Hemophagocytic Lymphohistiocytosis. Journal of Pediatric Hematology/Oncology 31:10, pages 778-781.
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Karin Beutel, Ute Gross-Wieltsch, Thomas Wiesel, Udo Zur Stadt, Gritta Janka & Hans-Joachim Wagner. (2009) Infection of T lymphocytes in Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in children of non-Asian origin. Pediatric Blood & Cancer 53:2, pages 184-190.
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Tatsuharu Ohno, Yo Ueda, Wataru Kishimoto, Kazue Arimoto-Miyamoto, Tomoharu Takeoka & Masaaki Tsuji. (2009) Epstein-Barr Virus-Induced Infectious Mononucleosis after Two Separate Episodes of Virus-Associated Hemophagocytic Syndrome. Internal Medicine 48:13, pages 1169-1173.
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Emiko Sato, Shouichi Ohga, Hiroshi Kuroda, Fumiaki Yoshiba, Miki Nishimura, Masayuki Nagasawa, Masami Inoue & Keisei Kawa. (2008) Allogeneic hematopoietic stem cell transplantation for Epstein–Barr virus‐associated T/natural killer‐cell lymphoproliferative disease in Japan. American Journal of Hematology 83:9, pages 721-727.
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Kris Ann P. Schultz, Joseph P. Neglia, Angela R. Smith, Hans D. Ochs, Troy R. Torgerson & Ashish Kumar. (2008) Familial hemophagocytic lymphohistiocytosis in two brothers with X-linked agammaglobulinemia. Pediatric Blood & Cancer 51:2, pages 293-295.
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Matthew Mischler, Geoffrey M. Fleming, Thomas P. Shanley, Lisa Madden, John Levine, Valerie Castle, Alexandra H. Filipovich & Timothy T. Cornell. (2007) Epstein-Barr Virus–Induced Hemophagocytic Lymphohistiocytosis and X-Linked Lymphoproliferative Disease: A Mimicker of Sepsis in the Pediatric Intensive Care Unit. Pediatrics 119:5, pages e1212-e1218.
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Shinsaku Imashuku. (2006) Etoposide‐related secondary acute myeloid leukemia (t‐AML) in hemophagocytic lymphohistiocytosis. Pediatric Blood & Cancer 48:2, pages 121-123.
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Jin-Seok LeeJin-Han KangGeon-Kook LeeHyeon-Jin Park. (2005) Successful Treatment of Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis with HLH-94 Protocol. Journal of Korean Medical Science 20:2, pages 209.
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S. Imashuku, T. Teramura, K. Konishi, A. Kikuchi, S. Tamura, Y. Maeda & H. Kuroda. (2004) Treatment of Epstein-Barr virus-associated peripheral T-cell lymphoma. British Journal of Dermatology 150:5, pages 1038-1040.
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Yun Li, Li Fu, Donna M. Gonzales & Ehud Lavi. (2004) Coronavirus Neurovirulence Correlates with the Ability of the Virus To Induce Proinflammatory Cytokine Signals from Astrocytes and Microglia. Journal of Virology 78:7, pages 3398-3406.
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S. Hasselblom, A. Linde & B. Ridell. (2004) Hodgkin's lymphoma, Epstein-Barr virus reactivation and fatal haemophagocytic syndrome. Journal of Internal Medicine 255:2, pages 289-295.
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Shinsaku Imashuku, Kikuko Kuriyama, Rika Sakai, Yoshitaka Nakao, Shin‐ichi Masuda, Norimasa Yasuda, Fumio Kawano, Kimikazu Yakushijin, Akiko Miyagawa, Taisei Nakao, Tomoko Teramura, Yasuhiro Tabata, Akira Morimoto & Shigeyoshi Hibi. (2003) Treatment of Epstein‐Barr virus‐associated hemophagocytic lymphohistiocytosis (EBV‐HLH) in young adults: A report from the HLH studyl center. Medical and Pediatric Oncology 41:2, pages 103-109.
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Ikuyo Ueda, Akira Morimoto, Tohru Inaba, Tomohito Yagi, Shigeyoshi Hibi, Tohru Sugimoto, Masahiro Sako, Fumio Yanai, Takashi Fukushima, Masahiko Nakayama, Eiichi Ishii & Shinsaku Imashuku. (2003) Characteristic perforin gene mutations of haemophagocytic lymphohistiocytosis patients in Japan. British Journal of Haematology 121:3, pages 503-510.
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Shinsaku Imashuku. (2002) Clinical features and treatment strategies of Epstein–Barr virus-associated hemophagocytic lymphohistiocytosis. Critical Reviews in Oncology/Hematology 44:3, pages 259-272.
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Shinsaku Imashuku, Nobuyuki Hyakuna, Tetsunori Funabiki, Koichiro Ikuta, Masahiro Sako, Asayuki Iwai, Takashi Fukushima, Satoshi Kataoka, Miharu Yabe, Kazuhiro Muramatsu, Urara Kohdera, Hisaya Nakadate, Katsuhiko Kitazawa, Yasunori Toyoda & Eiichi Ishii. (2002) Low natural killer activity and central nervous system disease as a high‐risk prognostic indicator in young patients with hemophagocytic lymphohistiocytosis. Cancer 94:11, pages 3023-3031.
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Shinsaku Imashuku, Tomoko Teramura, Kikuko Kuriyama, Junichi Kitazawa, Etsuro Ito, Akira Morimoto & Shigeyoshi Hibi. (2002) Risk of Etoposide-Related Acute Myeloid Leukemia in the Treatment of Epstein-Barr Virus—Associated Hemophagocytic Lymphohistiocytosis. International Journal of Hematology 75:2, pages 174-177.
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Urban Emmenegger, Peter J. Spaeth, Klaus A. Neftel, Shinsaku Imashuku, Tomoko Teramura, Eiichi Ishii, Naoko Kinugawa, Masahiko Kato, Masahiro Sako, Kikuko Kuriyama & Shigeyoshi Hibi. (2002) Intravenous Immunoglobulin for Hemophagocytic Lymphohistiocytosis?. Journal of Clinical Oncology 20:2, pages 599-601.
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Naoto Takahashi, Akihiko Chubachi, Masaaki Kume, Yoshiaki Hatano, Atsushi Komatsuda, Yoshinari Kawabata, Norimitsu Yanagiya, Yoshikazu Ichikawa, Akira B. Miura & Ikuo Miura. (2018) A Clinical Analysis of 52 Adult Patients With Hemophagocytic Syndrome: The Prognostic Significance of the Underlying Diseases. International Journal of Hematology 74:2, pages 209-213.
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Shinsaku ImashukuKikuko KuriyamaTomoko TeramuraEiichi IshiiNaoko KinugawaMasahiko KatoMasahiro SakoShigeyoshi Hibi. (2001) Requirement for Etoposide in the Treatment of Epstein-Barr Virus–Associated Hemophagocytic Lymphohistiocytosis. Journal of Clinical Oncology 19:10, pages 2665-2673.
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