噬血细胞综合征15例临床分析  

Clinical Analysis of 15 Patients With Hemophagocytic Syndrome

作　　者：曾淑华[1] 张星星[1] 梁海珊[2] 

机构地区：[1]广东医学院附属医院血液内科,湛江市524001 [2]广东医学院检验系

出　　处：《实用全科医学》2004年第3期195-196,共2页Applied Journal Of General Practice

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。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.

关 键 词：噬血细胞综合征 HPS 治疗 抗生素 血小板 诊断 

分 类 号：R557.4[医药卫生—血液循环系统疾病]