凝血功能障碍对儿童噬血细胞综合征预后的影响及意义

Prognostic significance of coagulation disorders in children with hemophagocytic syndrome

摘要:

目的 探讨凝血功能障碍对儿童噬血细胞综合征（HPS）预后的影响及意义。方法 回顾性分析35例HPS患儿的病因、临床特征、实验室检查结果及治疗转归情况。结果 35例HPS患儿经治疗后，27例存活，8例死亡。其中25例严格按照HLH-2004方案治疗，10例除未使用依托泊甙（VP-16）外，其余治疗方案均按HLH-2004治疗方案进行，使用VP-16治疗的患儿有效率（22/25，88%）高于未使用VP-16患儿（5/10，50%）（P<0.05）。死亡组血小板计数、纤维蛋白原水平和VP-16使用率均低于存活组，而活化部分凝血酶时间和凝血酶原时间均高于存活组（均P<0.05）。结论 凝血功能可做为评估HPS转归的一项指标，治疗过程中监测凝血功能，早期发现异常，及时纠正并严格按照HLH-2004方案正规治疗对改善HPS临床预后至关重要。

Abstract:

Objective To study the prognostic significance of coagulation disorders in children with hemophagocytic syndrome (HPS). Methods Thirty-five children with HPS were retrospectively studied to analyze the etiology, clinical characteristics, laboratory results and treatment outcomes. Results After treatment, 27 of the 35 HPS patients survived, and the other 8 cases died. All cases were treated according to the HLH-2004 protocol, but etoposide (VP-16) was not used in 10 of them. The response rate in patients who received VP-16 (22/25, 88%) was significantly higher than that in those not receiving VP-16 (5/10, 50%) (P<0.05). Compared with the survival group, the dead group had significantly lower platelet count, fibrinogen level, and VP-16 utilization rate (P<0.05) but significantly longer activated partial thromboplastin time and prothrombin time (P<0.05). Conclusions Coagulation function can be used as an indicator of disease outcome. It is essential for improving the clinical outcome of HPS to monitor the coagulation function during treatment, detect and correct abnormalities in time, and provide treatment strictly according to the HLH-2004 protocol.