淋巴瘤相关噬血细胞综合征临床特点  

Clinical characteristics of lymphoma associated hemophagocytic syndrome

作　　者：黎永谦[1] 陈素珊[1] 陈曼娜[1] 蔡宋浩[1] 叶俊彬[1] 杨琪玫[1] 杜则澎[2] 洪少杰[1] LI Yongqian;CHEN Sushan;CHEN Manna;CAI Songhao;YE Junbin;YANG Qimei;DU Zepeng;HONG Shaojie(Department of Hematology,Shantou Central Hospital,Guangdong Province,Shantou 515031,China;Department of Pathology,Shantou Central Hospital,Guangdong Province,Shantou 515031,China)

机构地区：[1]广东省汕头市中心医院血液科,广东汕头515031 [2]广东省汕头市中心医院病理科,广东汕头515031

出　　处：《中国医药导报》2019年第5期63-67,共5页China Medical Herald

基　　金：广东省汕头市科技计划项目(汕市财教[2014242号-47)

摘　　要：目的探讨淋巴瘤相关噬血细胞综合征的临床特点。方法对2012年1月～2017年6月广东省汕头市中心医院收治的18例淋巴瘤相关噬血细胞综合征患者的临床资料进行回顾性分析。结果 18例患者中有T细胞淋巴瘤13例,临床分期以中晚期占多数;不同性别淋巴瘤病理类型比较,差异无统计学意义(χ~2=0.628,P> 0.05),且淋巴瘤临床分期与发病年龄成正相关(r=0.765,P <0.05),年龄越大,淋巴瘤的临床分期越晚;淋巴瘤相关噬血细胞综合征相关基因检测发现有基因缺陷9例,其中UNC13D突变6例。治疗均参照噬血细胞综合征-94方案予激素联合依托泊苷改善患者的噬血症状后逐渐过渡至化疗。18例患者中有13例死亡,EB病毒感染及严重血小板减少是淋巴瘤相关噬血细胞综合征的死亡危险因素。结论淋巴瘤相关噬血细胞综合征临床表现多样,病死率高,多数患者存在EB病毒感染,早诊断、早治疗是关键。Objective To investigate the clinical characteristics of lymphoma associated hemophagocytic syndrome.Methods The clinical data of 18 patients with lymphoma associated hemophagocytic syndrome admitted to Shantou Central Hospital from January 2012 to June 2017 was analyzed retrospectively.Results Among 18 patients,there were 13 cases of patients with T cell lymphoma,and the main clinical stages were moderate and advanced stages.There was no significant difference of pathological types of lymphoma in different gender(χ^2=0.628,P>0.05).The clinical stage of lymphoma was positively correlated with the age of onset(r=0.765,P<0.05).The older the patients were,the later the clinical stage of lymphoma was.The related gene detection of lymphoma associated hemophagocytic syndrome showed that there were 9 cases of gene mutation,among which,6 cases had UNC13D mutations.According to hemophagocytic syndrome-94 regimen,all patients were given glucocorticoid and Etoposide to improve hemophagocytic symptoms and then gradually transferred to chemotherapy.13 of 18 patients died.EB virus infection and severe thrombocytopenia were the major causes of death of lymphoma related hemophagocytic syndrome.Conclusion Lymphoma related hemophagocytic syndrome has a wide variety of clinical manifestations and high mortality.Most patients have EB virus infection.Early diagnosis and treatment is the key.

关 键 词：淋巴瘤相关噬血细胞综合征 临床特点 EB病毒 基因突变 

分 类 号：R551.1[医药卫生—血液循环系统疾病]