Prognostic factors of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in children

Report of the Japan Histiocytosis Study Group

Kazuhiro Kogawa, Hiroki Sato, Takeshi Asano, Shoichi Ohga, Kazuko Kudo, Akira Morimoto, Shigeru Ohta, Hiroshi Wakiguchi, Hirokazu Kanegane, Megumi Oda, Eiichi Ishii

Research output: Contribution to journalArticle

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Abstract

Background: Despite several advances in the treatment of Epstein-Barr virus (EBV) in recent years, patients with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) do not always show satisfactory outcomes. We here conducted a nationwide survey in Japan to identify prognostic factors of EBV-HLH in children with this disease in an effort to improve the management and the outcomes of these patients. Procedure: Between January 2003 and June 2008, we enrolled 98 children younger than 18 years of age who were diagnosed with EBV-HLH. We then studied the clinical characteristics and laboratory findings at the time of diagnosis with the aim to identify prognostic factors for EBV-HLH. Results: The mean age of onset of EBV-HLH was 3.9±2.8 years. Most of our patients presented with fever, hepatosplenomegaly, lymphadenopathy, and hemophagocytosis of bone marrow. Sixty-two percent of patients showed T cell clonality, and 97% had EBV infection in either T or natural killer cells. Most patients (60%) were treated with a multi-agent chemotherapeutic regimen, including corticosteroid, etoposide, and cyclosporine. After initial treatment, 90.3% of patients were in remission, and 7 patients (8.2%) experienced recurrence of EBV infection. Among several prognostic factors, patients with both hyperbilirubinemia (>1.8mg/dl) and hyperferritinemia (>20,300ng/ml) at the time of diagnosis had significantly poorer outcomes than those with low serum bilirubin and ferritin levels. Conclusions: These findings suggest that the therapeutic strategy for children with EBV-HLH could be tailored according to the laboratory findings at diagnosis. Pediatr Blood Cancer 2014;61:1257-1262.

Original languageEnglish
Pages (from-to)1257-1262
Number of pages6
JournalPediatric Blood and Cancer
Volume61
Issue number7
DOIs
Publication statusPublished - Jan 1 2014

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Histiocytosis
Hemophagocytic Lymphohistiocytosis
Human Herpesvirus 4
Japan
Epstein-Barr Virus Infections
Natural Killer T-Cells
Hyperbilirubinemia
Etoposide
Ferritins
Age of Onset
Bilirubin
Cyclosporine
Adrenal Cortex Hormones
Fever
Therapeutics
Bone Marrow
T-Lymphocytes
Recurrence

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology

Cite this

Prognostic factors of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in children : Report of the Japan Histiocytosis Study Group. / Kogawa, Kazuhiro; Sato, Hiroki; Asano, Takeshi; Ohga, Shoichi; Kudo, Kazuko; Morimoto, Akira; Ohta, Shigeru; Wakiguchi, Hiroshi; Kanegane, Hirokazu; Oda, Megumi; Ishii, Eiichi.

In: Pediatric Blood and Cancer, Vol. 61, No. 7, 01.01.2014, p. 1257-1262.

Research output: Contribution to journalArticle

Kogawa, K, Sato, H, Asano, T, Ohga, S, Kudo, K, Morimoto, A, Ohta, S, Wakiguchi, H, Kanegane, H, Oda, M & Ishii, E 2014, 'Prognostic factors of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in children: Report of the Japan Histiocytosis Study Group', Pediatric Blood and Cancer, vol. 61, no. 7, pp. 1257-1262. https://doi.org/10.1002/pbc.24980
Kogawa, Kazuhiro ; Sato, Hiroki ; Asano, Takeshi ; Ohga, Shoichi ; Kudo, Kazuko ; Morimoto, Akira ; Ohta, Shigeru ; Wakiguchi, Hiroshi ; Kanegane, Hirokazu ; Oda, Megumi ; Ishii, Eiichi. / Prognostic factors of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in children : Report of the Japan Histiocytosis Study Group. In: Pediatric Blood and Cancer. 2014 ; Vol. 61, No. 7. pp. 1257-1262.
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AU - Ohga, Shoichi

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AU - Morimoto, Akira

AU - Ohta, Shigeru

AU - Wakiguchi, Hiroshi

AU - Kanegane, Hirokazu

AU - Oda, Megumi

AU - Ishii, Eiichi

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