淋巴瘤相关性噬血细胞综合征14例临床分析  

Clinical Analysis of 14 Patients with Lymphoma Associated Hemophagocytic Syndrome

作　　者：王晶石[1] 王昭[1] 吴林[1] 田莉萍[1] 陈晳 

机构地区：[1]首都医科大学北京友谊医院血液科,北京100050

出　　处：《中国实验血液学杂志》2009年第5期1352-1355,共4页Journal of Experimental Hematology

基　　金：首医基础-临床合作课题资助,编号2007JL17

摘　　要：本研究分析淋巴瘤相关性噬血细胞综合征(LAHS)患者的临床特征和诊疗手段,为进一步提高LAHS诊断率、选择及时有效的治疗方案提供临床依据。收集2005年6月至2008年5月经首都医科大学附属北京友谊医院确诊的LAHS患者14例,回顾性分析其原发病、临床特征、实验室检查指标、治疗与转归,探讨疾病特点,并比较LAHS患者在确诊前后各项诊断标准的符合情况,了解各项诊断指标在疾病诊断中的意义。14例患者确诊后均采用FDIg方案(氟达拉滨,甲基强的松龙,丙种球蛋白)治疗,观察治疗效果。结果表明,14例LAHS患者的NK细胞活性、sCD25水平在疾病早期100%出现异常,而骨髓中噬血现象仅见于不到40%的患者,即使在确诊后,也只有不到50%患者出现了噬血。经FDIg方案治疗的14例患者其中9例(64.3%)病情好转;5例(35.7%)死亡。结论:NK细胞活性、血清sCD25水平的检测对于LAHS的早期诊断可能具有重要的意义,而噬血现象在疾病的诊断中并不一定起主要作用。以氟达拉滨联合激素和免疫球蛋白的治疗方案(FDIg方案)为早期有效治疗LAHS提供新思路。The study was aimed to investigate the clinical characteristics, diagnosis and therapy of patients with lymphoma associated hemophagocytic syndrome （LAHS） so as to provide the clinical basis for improving its recognition and giving effective therapy. The clinical data of 14 patients with LAHS in Beijing Friendship Hospital, Capital Medical University during the period from June 2005 to May 2008 were collected, the informations including primary diseases, clinical manifestations, laboratory findings, therapy and outcome were analyzed retropectively, the coincidence of each diagnostic index was compared before and after diagnosis. All 14 patients were given therapeutic regimens containing fludarabine, methylprednisolone and gammaglobulin （ FDIg ） after final diagnosis. The results indicated that 100% patients had abnormal chemges on NK cell activity and sCD25 level in semm, but hemophagocytosis in less than 40% patients at early stage was found in bone marrow. Even after confirmed diagnosis of the disease, the percentage of patients with hemophagocytosis was not up to 50%. 9 out of the 14 patients had a good prognosis after treatment, and the other 5 patients died. It is concluded that the detection of NK cell activity and sCD25 level in serum may be valuable for the early diagnosis of LAHS, the hemophagocytosis is not necessary for the diagnosis of LAHS. Fludarabine combined with methylprednisolone and gamma globulin may provide a new strategy for LAHS therapy.

关 键 词：淋巴瘤 淋巴瘤相关噬血细胞综合征 氟达拉滨 

分 类 号：R733.1[医药卫生—肿瘤] R557.4[医药卫生—临床医学]