Tsuchiya Children's Hospital, Kuki, Saitama, Japan; HLH/LCH Committees in the Japanese Society of Pediatric Hematology, Tokyo, Japan.
Pediatr Blood Cancer. 2014 Jul;61(7):1257-62. doi: 10.1002/pbc.24980. Epub 2014 Feb 17.
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.
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.
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 (>1.8 mg/dl) and hyperferritinemia (>20,300 ng/ml) at the time of diagnosis had significantly poorer outcomes than those with low serum bilirubin and ferritin levels.
These findings suggest that the therapeutic strategy for children with EBV-HLH could be tailored according to the laboratory findings at diagnosis.
尽管近年来在治疗 EBV（Epstein-Barr 病毒）方面取得了一些进展，但 EBV 相关噬血细胞性淋巴组织细胞增多症（EBV-HLH）患者的治疗效果并不总是令人满意。因此，我们在日本进行了一项全国性调查，旨在确定该病患儿 EBV-HLH 的预后因素，以期改善这些患者的治疗效果。
2003 年 1 月至 2008 年 6 月期间，我们共纳入 98 例年龄小于 18 岁的 EBV-HLH 患儿。研究了他们在诊断时的临床特征和实验室检查结果，旨在确定 EBV-HLH 的预后因素。
EBV-HLH 的平均发病年龄为 3.9±2.8 岁。大多数患儿表现为发热、肝脾肿大、淋巴结肿大和骨髓噬血现象。62%的患儿存在 T 细胞克隆性，97%的患儿存在 T 细胞或自然杀伤细胞 EBV 感染。大多数患儿（60%）接受了包括皮质类固醇、依托泊苷和环孢素在内的多药化疗方案。初始治疗后，90.3%的患儿病情缓解，7 例（8.2%）患儿 EBV 感染复发。在多个预后因素中，诊断时存在高胆红素血症（>1.8mg/dl）和高铁蛋白血症（>20,300ng/ml）的患儿与低胆红素和铁蛋白水平患儿相比，预后明显较差。
这些发现表明，可以根据诊断时的实验室检查结果为 EBV-HLH 患儿制定个体化的治疗策略。
