Hybrid血液净化技术治疗儿童噬血细胞综合征  

Application of Hybrid blood purification technique for the treatment of hemophagocytic syndrome in children

作　　者：徐洪波[1] 干毅[1] 黄婷芸 田茂强[1] 路健[1] 陈艳[1] Xu Hongbo;Gan Yi;Huang Tingyun;Tian Maoqiang;Lu Jian;Chert Yah(Department of Pediatrics,Affiliated Hospital of Zunyi Medical University,Zunyi Guizhou 563099,China)

机构地区：[1]遵义医学院附属医院小儿内二科,贵州遵义563099

出　　处：《遵义医学院学报》2018年第4期481-485,共5页Journal of Zunyi Medical University

摘　　要：目的观察杂交血液净化技术在儿童噬血细胞淋巴组织细胞增生症(hemophagocytic lymphohistiocytosis,HLH)中的治疗效果。方法收集2015年6月至2017年6月遵义医学院附属医院PICU收治的HLH,且行血液净化治疗的3例患儿的临床资料。明确诊断HLH后按HLH-2004方案治疗无效或出现合并症时进行床旁血浆置换(plasma exchange,PE)+持续血液净化(模式为CVVHDF或CVVH)治疗。收集患者年龄、触发因素、临床资料、实验室检查及基因检测结果,比较患者血液净化治疗前后临床症状及相关生化指标变化。出院后随访有无复发。结果①3例患儿,最小10月,最大72个月;②1例为原发性HLH,另2例为继发性HLH(EB病毒相关性),均为感染触发;③临床症状及体征:发热(3/3)、黄疸(3/3)、尿少(2/3)、浮肿(2/3)及肝脾肿大(3/3)等;(4)实验室检查三系下降(3/3)、纤维蛋白原降低(3/3)、高甘油三酯血症(2/3)、血清铁蛋白增高(3/3)、NK细胞活性下降(3/3)、组织细胞噬血现象(1/3)等;(5)3例患者行基因检测仅1例发现UNC13D基因Exon14突变:c.1228A>C(p.Ile410Leu)。经持续血液净化(时间56～136 h)+血浆置换治疗后3例患者临床症状改善,体温正常、浮肿及黄疸消退、肝脾回缩,血常规三系回升,铁蛋白、IL-6、ALT和LDH显著降低,最终好转出院。至最后1次随访(2017年12月),3例患者各项指标正常,均无复发。结论 HLH是一种临床综合征,对于重症患者,可加用Hybrid血液净化技术辅助治疗。Objective To analyze the effects of blood purification technique on treating children with hemophagocytic lymphohistiocytosis（HLH）. Methods Retrospective chart view of three patients with HLH in the Affiliated Hospital of Zunyi Medical University during June 2015 to 2017 was conducted. All the patients were treated with bedside plasma exchange（PE） and continuous blood purification（CVVHDF or CVVH） when the HLH-2004 regimen was ineffective or comorbidities were occurred. The patients＇ clinical data were collected,including age,triggering factors,clinical symptoms,signs,laboratory examination,and gene results. The variations in clinical symptoms and related biochemical indicators were compared which were observed before and after blood purification treatment. The recurrence was followed up after discharging. Results Among the three patients,the youngest patient was 10 months,the oldest was 72 months. One patient was diagnosed with primary HLH,and the other two were secondary HLH（EB virus-associated）. All the patients were triggered by the infection. Clinical signs and symptoms： fever（3/3）,oliguria（2/3）,edema（2/3）,jaundice（3/3）,and liver Splenomegaly（3/3） and so on. Laboratory Findings： pancytopenia（3/3）,hypofibrinogenemia（3/3）,hypertriglyceridemia（2/3）,hyperferroproteinemia（3/3）,NK cell activity decreased（3/3）,tissue cell hemophagocytosis（1/3）,etc. Genetic test was performed in all patients. Only one patient had UNC13 D Exon14 mutation： c. 1228 A C（p. Ile410 Leu）.After continuous blood purification（56 - 136 h） and plasma exchange,the three patients＇ clinical symptoms were improved. It was observed that body temperature returned to normal,edema and jaundice were subsided,hepatosplenomegaly was retracted,blood routine was recovered,and the levels of SF,IL-6,ALT and LDH were significantly reduced. Finally,all the patients could be discharged. At the last follow-up visit（December2017）,all the indicators were normal and there was no recurre

关 键 词：儿童 噬血细胞综合征 血液净化 

分 类 号：R725.5[医药卫生—儿科]