摘要
目的:探讨噬血细胞综合征患者的临床特点、治疗及预后。方法:分析18例患者临床症状、体征、实验室资料、原发病、治疗及转归。结果:18例患者主要表现为发热(94.4%)、肝脾淋巴结肿大(77.8%)、外周血细胞减少(>61.1%)、肝功能异常(88.9%);经针对原发病及采用化疗和免疫疗法治疗后3例治愈,6例好转,4例放弃治疗,5例死亡。结论:小儿噬血细胞综合征临床病情凶险,病死率高,早期诊断和及时治疗对预后十分关键。
Objectives： To investigate the clinical characteristics, treatment and prognosis of children patients with hemophagocytic syndrome （HPS）. Methods： The clinical symptoms, signs, laboratory data, primary diseases and prognosis of 18 children patients with HPS were analyzed retrospectively. Results： The main clinical manifestations were fever（94.4% ）, hepatosplenomegaly and swelling of lymph nodes （.77.8%）, pancytopenia （〉61.1%）, liver disfunction （88.9%）. The primary diseases were treated. In some cases, induction chemotherapy and immunomodulatory therapy were also adopted. 3 patients recovered, 6 improved, 4 gave up and 5 patients died. Conclusions： Children patients with HPS often undergo an aggressive clinical course resulting in a poor prognosis. Early diagnosis and promptly treatment is the key point for prognosis.
出处
《岭南急诊医学杂志》
2006年第1期29-30,共2页
Lingnan Journal of Emergency Medicine
关键词
噬血细胞综合征
临床特点
治疗
预后
hemophagocytic syndrome
clinical characteristics
treatment
prognosis
