成人噬血细胞综合征8例临床分析

Clinical analysis of 8 adult patients with hemophagocytic syndrome

目的:探讨噬血细胞综合征(HPS)的病因、临床特点、治疗及预后。方法:对8例噬血细胞综合征的临床资料进行回顾性分析。结果:8例患者原发病以淋巴瘤居多。主要临床表现以持续高热(100%)、肝脾肿大(83%)、出血症状最多,血清LDH显著增高,肝功损害,血清铁蛋白异常增高,甘油三酯增高,三系细胞减少,凝血功能异常、骨髓中可见噬血现象。予以联合化疗、大剂量丙种球蛋白(IVIG)、激素冲击治疗等有一定疗效。死亡7例,存活1例。结论:成人HPS病因多以淋巴瘤为主,临床过程凶险,预后差,死亡率高,联合化疗、大剂量IVIG、激素冲击治疗有一定疗效。

Objective：To investigate the causes,clinical features, treatment and prognosis of hemophagocytic syndrome. Methotls：A retrospective analysis of clinical data of 8 patients with hemophagocytic syndrome. Results：Among the 8 patients,lymphoma was the most common initiating disease. The most common clinical features were persistent fever （ 100% ）, hepatosplenomegaly （ 83% ）, bleeding symptoms. Increased serum LDH, hepatic dysfunction, abnormal increase of serum ferritin, higher triglycerides, peripheral cytopenia, coagulation dysfunction, hemophagocytic phenomenon in the bone marrow. Chemotherapy, high - dose IVIG and ethylprenisolone are effective therapy. Of 8 cases 7 died, one case survived . Condusion：Lymphoma is the most cause of adult HPS, with extremely poor prognosis and high mortality. Chemotherapy, high - dose IVIG and ethylprenisolone are effective therapy.