Department of Clinical Haematology, Children Hospital, Ho-Chi-Minh City, Vietnam.
Br J Haematol. 2010 Jan;148(2):301-10. doi: 10.1111/j.1365-2141.2009.07957.x. Epub 2009 Oct 27.
Haemophagocytic lymphohistiocytosis (HLH) is a fatal haematological disorder with diverse aetiology. This prospective study was undertaken to characterize HLH cases in Vietnamese children. Clinical and laboratory data, genetic analyses and outcome of the HLH patients were analysed. A total of 33 patients were enrolled from March 2007 to December 2008, with a median age of 3 years. Mutations of the SH2D1A (SAP) and PRF1 genes were detected in one patient, respectively. The virus association was high, up to 63.6% (21/33), including Epstein-Barr virus (19/33), cytomegalovirus (2/33) and dengue virus (2/33). Five patients had malignant lymphoma and two had autoimmune diseases. Twenty-eight patients were treated according to the HLH-2004 protocol. The first response rate was 64.3% (18/28), with an early death rate of 35.7% (10/28). High levels of interferon-gamma, interleukin-10, MIG and interferon-inducible protein-10 (IP-10) were associated with early mortality (P < 0.05). Reactivation among the responders was high (9/18) and the uneventful resolution was low (3/18) after a median follow-up of 35 weeks. In conclusion, the majority of HLH cases are associated with virus infections in Vietnamese children. Familial HLH is rare. The frequent reactivation and high mortality demands a more appropriate therapeutic regimen in tropical areas like Vietnam.
噬血细胞性淋巴组织细胞增生症（HLH）是一种具有多种病因的致命血液系统疾病。本前瞻性研究旨在描述越南儿童 HLH 病例的特征。分析了 HLH 患者的临床和实验室数据、基因分析和结局。2007 年 3 月至 2008 年 12 月共纳入 33 例患者，中位年龄为 3 岁。分别在 1 例患者中检测到 SH2D1A（SAP）和 PRF1 基因突变。病毒相关性高，达 63.6%（21/33），包括 EBV（19/33）、CMV（2/33）和登革热病毒（2/33）。5 例患者有恶性淋巴瘤，2 例有自身免疫性疾病。28 例患者根据 HLH-2004 方案进行治疗。初次反应率为 64.3%（18/28），早期死亡率为 35.7%（10/28）。干扰素-γ、白细胞介素-10、MIG 和干扰素诱导蛋白-10（IP-10）水平高与早期死亡相关（P<0.05）。在中位随访 35 周后，应答者中再激活率高（9/18），无事件缓解率低（3/18）。总之，大多数 HLH 病例与越南儿童的病毒感染有关。家族性 HLH 罕见。在像越南这样的热带地区，频繁再激活和高死亡率需要更合适的治疗方案。
