噬血细胞综合征患儿血液净化治疗现况调查

A survey on the current status of blood purification treatment in children with hemophagocytic lympho-histiocytosis

目的 了解我国噬血细胞综合征[噬血性淋巴组织细胞增生症（HLH）]患儿行血液净化治疗的现状.方法 中国医师协会儿科医师分会血液净化专业委员会专家制定《噬血细胞综合征登记表》,对国内部分医院近期行血液净化治疗的HLH患儿进行回顾性调查分析.结果 （1）病例构成：15家医院提供66例经血液净化治疗的HLH病例,其中18例来自肾脏科,其余来自PICU;男42例（63.6％）,女24例（36.4％）,男：女1.83：1,中位年龄3.23岁（0.7-13.0岁）.血浆置换（plasma exchange,PE）组29例,PE＋连续性肾脏替代治疗（continuous renal replacement therapy,CRRT）29例,CRRT 5例（统称CRRT组）;PE＋血液灌流组2例,PE＋血液透析组1例;（2）病因构成：感染60例（91.0％）、淋巴瘤3例（4.5％）、风湿性疾病3例（4.5％）;（3）临床表现：CRRT组器官衰竭多于PE组,两组存活病例治疗后临床表现均缓解;（4）实验室指标：血液净化治疗前PE组与CRRT组患儿血细胞明显减少,肝肾功能、凝血功能损害明显,治疗后均缓解,铁蛋白在PE组下降幅度更显著（P〈0.05）,C-反应蛋白在CRRT组下降幅度较大;（5）转归：应答39例（59.1％）;存活39例（59.1％）,死亡及退院27例（40.9％）.结论 HLH患儿的血液净化模式以PE和CRRT为主,治疗后大部分患儿临床表现及实验室指标缓解,血液净化治疗HLH的指征及时机待进一步完善.

Objective To study the current status of blood purification treatment in children with he-mophagocytic lymphohistiocytosis（HLH）. Methods Experts of the Pediatric Blood Purification Specialist Committee collected and analyzed the data of hospitalized children with HLH undergoing blood purification treatment in some hospitals in China. Results （1）Case composition：there were 66 cases of HLH treated by blood purification in 15 hospitals,18 of which were from the Department of Nephrology and the others from PICU;42 males （63. 6%）,24 females （36. 4%）,males：females was 1. 83：1. The median age was 3. 23 years old （0. 7-13. 0 years old）. Twenty-nine cases in plasma exchange （PE） group,29 cases in PE＋ con-tinuous renal replacement therapy （ CRRT） group,5 cases in CRRT group,2 cases in PE＋ hemoperfusion group,and 1 case in PE ＋ hemodialysis group;（2） Etiology： 60 cases （91. 0%） with infection,3 cases （4. 5%） with lymphoma, 3 cases （4. 5%） with rheumatic diseases;（3） Clinical manifestation：the cases of organ failure in the CRRT group were more than PE group, and the clinical manifestation of the survivingcases in both groups got better after treatment;（4） Laboratory indicators：blood cells were significantly lower than normal level in the two groups,and liver,renal,coagulation were disfunctioned. After treatment,all indi-cators were more closer to normal level. The decrease of serum ferritin in PE group was more significant （P〈0. 05）. C-reactive protein decreased more in the CRRT group than that in the PE group. （5）Outcome：responded：39 cases （ 59. 1%）; survival：39 cases （ 59. 1%） , died and discharged from hospital：27 cases （40. 9%）. Conclusion The main blood purification modes of HLH are PE and CRRT. After treatment,the clinical manifestation and laboratory indicators of most children are better. The indications and the timing for blood purification treatment of HLH need further confirmed.