噬血细胞性淋巴组织细胞增生症前瞻性研究

Prospective Study of Hemophagocytic Lymphohistiocytosis in Children

目的探讨儿童噬血细胞性淋巴组织细胞增生症(HLH)的临床特点及预后影响因素。方法2004年1月-2007年5月本院收治HLH患儿64例。男43例,女21例;中位年龄4岁3个月。除诊断所需检测项目外,化疗前常规行头部CT或MRI检测,行脑脊液生化及常规分类、爱泼斯坦病毒(EBV)、巨细胞病毒、单纯疱疹病毒、柯萨奇病毒、埃可病毒抗体水平检测;并定期监测上述指标以评估疗效,对所有HLH采用统一的免疫化疗方案。结果1.化疗2周后,1例死亡,4例于化疗后8周放弃治疗未评估疗效,余59例均临床有效,其中21例随后因各种原因放弃治疗,32例进入停药观察期,其中3例停药后1个月内复发。2.患儿64例中EBV相关HLH(EBV-HLH)患儿47例(73.4%),29例疗效差者中24例(82.7%)为EBV-HLH。3.有60例(93.8%)患儿中枢神经系统受累,且影像学改变先于临床症状的出现。结论1.该组研究中多数患儿更倾向于继发HLH诊断。2.EBV-HLH是我国儿童HLH的主要类型,也是重要的预后不良因素之一。3.HLH患儿中枢神经系统受累的发病率高,预后差,但通过早期积极的治疗是可逆的。4.对HLH患儿确诊后应常规行头颅MRI检查。

Objective To explore the clinical characteristics and influential factors on prognosis of hemophagocytic lymphohistiocytosis （HLH） in children. Methods Sixty - four children with HLH were registered in this study group from Jan. 2004 to May 2007. Of these, twenty - one cases were girls and 43 cases were boys ; the median age at diagnosis was 4 years and 3 months. Apart from items needed by the diagnosis, brain computer tomography （CT） or magnetic resonance imaging （ MRI）, cerebrospinal fluid analysis and antibodies of Epstein - Barr virus （EBV）, cytomegalovirus, herpes simplex virus, Coxsackie virus, echo virus were detested prior chemotherapy routinely. All examinations above mentioned were monitored regularly. All patients were adopted the same immunochemotherapy protocol. Results 1. One child died and 4 children gave up therapy in 8 weeks after therapy without assessment. The rest 59 children showed improvement, and of these 21 children gave up therapy later on because of various reasons. Thirty - two children were off - therapy, and among them 3 children relapsed in 1 month. 2. Altogether 47 children （73.4%） had EBV infection, also EBV associated HLH （ EBV - HLH）. Among the 29 children with unfavourable result （ death and relapse）, 24 children （ 82.7 % ） were EBV - HLH. 3. Altogether 60 children （93.8%） had central nervous system（CNS） involvement and neuroradiological changes precede clinical manifestations. Conclusions 1. Majority of children in this study were prone to be secondary. HLH. 2. EBV - HLH is the main type of the secondary HLH in our country, and also is one of critical unfavourable prognosis factors. 3. CNS involvement in HLH has a very high frequency and poor prognosis. But that nonetheless there is still hope to recover completely as long as proper chemotherapy treatment is applied at an early stage. 4. Now that brain MRI changes can precede neurological symptoms, all children should have a MRI performed routinely from diagnosis.