儿童噬血细胞综合征38例临床分析

摘要目的：探讨噬血细胞综合征患儿的临床特征、治疗及预后。方法：对38例噬血细胞综合征患儿的Il盏床症状、体征、试验检查结果、治疗及预后，进行回顾性总结与分析。结果：38例患儿主要表现为发热、肝脾淋巴结肿大、外周血细胞减少、铁蛋白升高、凝血功能异常；经针对性治疗后，治愈3例，好转20例，疗效不佳自动出院9例，死亡6例。结论：噬血细胞综合征病因复杂，临床表现多种多样，病情凶险，预后较差，旱期诊断及治疗对预后十分关键。

Clinical Analysis of 38 Case of Childhood Hemophargocytic Syndrome

Objective： To investigate the clinical characteristics, treatments and prognosis of children with hemophargocytic synd- rome. Methods： Aretrospective summary was performed, and the clinical symptoms, signs, laboratory test results, treatments and progno- sis of 38 cases of childhood hemophargocytic syndrome were analyzed. Results： The main manifestations included fever, enlargement of liver and spleen lymph nodes, reduction of peripheral blood cells, increase offerritin, coagulant function abnormality. After the specific treatments, 3 patients cured, 20 patients improved,9 patients discharged voluntarily due to poor efficacy, 6 patients died. Conclusion： Hemophagocytic syndrome has complicated causes, diverse clinical manifestations, dangerous conditions and poor prognosis. Early diagnosis and treatment are very critical to the prognosis.