【摘要】 目的　了解噬血细胞综合征 (HPS)患者的临床特点、治疗及预后。方法　分析 15例患者临床症状、体征、实验室资料、原发病、治疗及转归。结果　 15例患者主要表现为发热、肝脾淋巴结肿大、外周血细胞减少、黄疸及低钠血症 ,6例中性粒细胞碱性磷酸酶 (NAP)活性显著增强 ,经针对原发病及采用ED方案治疗后 7例治愈 ,5例好转 ,2例死亡。结论　血液系统肿瘤HPS多见于恶性淋巴瘤 ,免疫调节系统异常在其发生中具有重要作用。更多还原

【Abstract】 Objectives To investigate the clinical features, treatment and prognosis of hemophagocytic syndrome (HPS). Methods The clinical symptoms, signs, laboratory data, primary diseases and prognosis of 15 patients with HPS were analyzed. Results The main clinical manifestations were fever, hepatosplenomegaly, swelling of lymph nodes, pancytopenia, jaundice and hyponatremia. The activity of neutrophil alkaline phosphatase (NAP) increased markedly in 6 cases. In all cases, the primary diseases were treated with ED project, 7 patients recovered, 5 patients improved and 2 patients died. Conclusions HPS mainly occurs with malignant lymphoma. The dysfunction of immunoloregulation may be one important mechanism in occurrence of HPS.更多还原