Objective: To investigate the difference in the therapeutic effect of plasma exchange and continuous renal replacement therapy (PE+CRRT) combined with chemotherapy in the treatment of children with severe Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) and non-EBV-HLH.
Methods: The clinical data of 21 cases of all children with severe HLH treated by PE+CRRT combined with chemotherapy from January 2017 to January 2020 were collected and retrospectively analyzed. According to the presence of EBV infection, the children were divided into EBV+ group and EBV- group. The differences of the observation indexes between the children in the two groups and the improvement of the observation indexes of each group before and after treatment were compared.
Results: Among the 21 children, 14 were divided into the EBV+ group and 7 were divided into the EBV- group. There was no difference in age, sex and the number of organ damage between the children in the two groups (P>0.05). Duration of PE+CRRT was longer in the EBV+ group as compared with the EBV- group (P<0.05). Before treatment, the ANC in the EBV+ group was lower than that in the EBV- group (P<0.05), and there was no significant difference in the other observation indexes between the two groups (P>0.05). After treatment, Hb, Fib, APTT, SF, ALT, AST, LDH, Alb, CHE, TBil and TBA of the children in the EBV+ group were significantly improved as compared with those before treatment (P<0.05), but ANC, PLT, TG showed not improve (P>0.05); Fib, APTT, SF, LDH, Alb, and CHE in the EBV- group were significantly as improved compared with those before treatment (P<0.05), while the ANC, PLT, Hb, TG, ALT, AST, TBil, and TBA were not improved (P>0.05). After treatment, the differences of Fib and SF in the children between the EBV+ group and the EBV- group were statistically significant (P<0.05), and there was no significant difference in the other observation indexes of the children between the two groups (P>0.05). Compared with the children before treatment, EBV-DNA in the EBV+ group were decreased significantly in 2-4 weeks after treatment (P<0.05). After PE+CRRT combined with chemotherapy, the overall survival rate of the children with severe HLH was 66.7%, and there was no significant difference in overall survival rate between EBV+ group and EBV- group (P>0.05).
Conclusion: PE+CRRT combined with chemotherapy can reduce serum ferritin quickly, then improve organ function, and increase the overall survival rate of severe HLH, and it is a good effect on children with severe EBV-HLH and non-EBV-HLH.
题目: PE+CRRT联合化学免疫疗法治疗儿童重型EBV-HLH和非EBV-HLH的疗效观察.
目的: 探讨血浆置换与连续性肾脏替代疗法(PE+CRRT)联合化学免疫疗法在儿童重型EB病毒相关噬血细胞综合征（EBV-HLH）和非EBV-HLH中疗效的差异.
方法: 收集并回顾性分析2017年1月至2020年1月所有接受PE+CRRT联合化学免疫疗法的21例重型HLH患儿的临床资料，根据是否存在EBV感染，将患儿分为EBV+组和EBV-组，比较两组间各观察指标的差异及各组治疗前后各观察指标的改善情况.
结果: 21例患儿中，EBV+组14例，EBV-组7例，两组年龄、性别及器官损害数无差异（P>0.05）。与EBV-组相比，EBV+组行PE+CRRT的持续时间更长（P<0.05)。治疗前，EBV+组ANC较EBV- 组明显降低（P<0.05），其余观察指标两组间比较差异无统计学意义（P>0.05）。治疗后，EBV+组Hb、Fib、APTT、SF、ALT、AST、LDH、Alb、CHE、TBil及TBA较治疗前均可见明显改善（P<0.05），ANC、PLT、TG与治疗前相比未见改善（P>0.05）；EBV- 组Fib、APTT、SF、LDH、Alb、CHE较治疗前明显改善（P<0.05），ANC、PLT、Hb、TG、ALT、AST、TBil、TBA与治疗前相比未见改善（P>0.05）。治疗后，EBV+组和EBV-组间Fib、SF差异具有统计学意义（P<0.05），两组间其余观察指标差异无统计学意义（P>0.05）。与治疗前相比，治疗后2-4周，EBV+组EBV-DNA显著降低(P<0.05)。接受PE+CRRT联合化学免疫疗法后，重型HLH患儿总生存率为66.7%，EBV+组和EBV- 组患儿总生存率差异无统计学意义（P>0.05）.
结论: PE+CRRT联合化学免疫疗法可快速降低血清铁蛋白、改善器官功能，提高重型HLH总生存率，且对儿童重型EBV-HLH和非EBV-HLH均有较好的疗效，是治疗HLH的有效方法.