20例成人嗜血细胞综合征的临床分析

Clinical analysis of twenty adult patients with hemophagocytic syndrome

目的探讨成人嗜血细胞综合征(HPS)的病因、临床特点、诊断、治疗及预后。方法回顾性分析2011年3月-2014年5月本院20例嗜血细胞综合征患者的临床资料。结果临床表现:20名患者初治时均有发热(100%),其中黄疸5例(25%),脾大19例(95%),肝脾大7例(35%),淋巴结肿大6例(30%),浆膜腔积液7例(35%);实验室检查:20例均有两系或三系血细胞减少、肝功损害,凝血功能异常,19例(95%)LDH增高,16例(80%)铁蛋白增高,20例(100%)均于骨髓中查见嗜血现象。7例(35%)最终确定为淋巴瘤,6例(30%)系EB病毒感染所致,7例(35%)原因不明。治疗后,3例好转,17例死亡。结论成人HPS多起病凶险,进展迅速,死亡率高,目前尚无有效治疗方案,其治疗手段有待进一步研究。

Objective To study the etiology,clinical manifestations,diagnosis,treatment and prognosis of hemophagocytic syndrome（ HPS）. Methods Twenty cases of patients with HPS from March 2011 to May 2014 were analyzed retrospectively. Results All patients presented fever（ 100. 0%） at first interview,5 had jaundice（ 25%）,19 had splenomegaly（ 95%）,7 had hepatosplenomegaly（ 35%）,6 had enlarged lymph nodes（ 30%）,and 7 had serous cavity effusion（ 35%）. In addition,laboratory test results showed that 20 patients had pancytopenia（ 100%）,impaired liver function（ 100%）,hematostatic abnormality（ 100%）,and hemophagocytosis in bone marrow（ 100. 0%）. At the same time,19 patients had increased lactate dehydrogenase（ 95%）,16 had elevated ferritin（ 80%）. 7 HPS cases（ 35%） were caused by lymphoma,6 were caused by EB virus infection（ 30%）,and the rest of the cases（ 35%） was idiopathic. Three patients recovered and 17 died after treatment. Conclusion Adult HPS is an disease with complex clinical presentation,an aggressive course,and a high mortality rate. There is no effectual treatment for HPS currently. The therapeutic methods for HPS should be further studied.