【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。更多还原

【Abstract】 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.更多还原