12例继发性噬血细胞性淋巴组织细胞增多症疾病再活动患者临床分析  

Clinical analysis of 12 patients with reactivated secondary hemophagocytic lymphohistocytosis

作　　者：陈纬凤[1] 仇红霞[1] 王菱菱[1] 胡昳歆[1] 张蔚[1] 许戟[1] 张苏江[1] 张晓艳[1] 李建勇[1] 

机构地区：[1]南京医科大学第一附属医院江苏省人民医院血液科,南京210029

出　　处：《临床血液学杂志》2013年第3期289-291,294,共4页Journal of Clinical Hematology

基　　金：国家自然科学基金面上项目(No:81070456;81270652);江苏省"六大高峰人才"(No:2010-WS-019)

摘　　要：目的:探讨继发性噬血细胞性淋巴组织细胞增多症(sHLH)疾病再活动患者的临床特点。方法:对12例sHLH疾病再活动患者进行回顾性分析。结果:12例sHLH患者疾病再活动后临床表现及实验室检查指标主要表现为再次出现初诊时的异常。挽救治疗则采用个体化治疗方案。sHLH患者疾病再活动主要发生在初始治疗后6周左右。4例患者存活,平均生存时间为(182.50±40.52)d;8例患者死亡,总体病死率为67%,死亡组患者的平均生存时间为(14.25±7.09)d。结论:sHLH患者疾病再活动后病情进展迅速,病死率高。患者病情再活动后需重新进行全面的病情评估,再次提高激素用量可能是有效的治疗方法之一。Objective：This study was aimed to investigate the clinical characteristics of 12 patients with reacti- vated secondary hemophagocytic lymphohistocytosis （sHLH）. Method： Clinical characteristics of 12 patients with reactivated sHLH were analyzed retrospectively. Result： The clinical manifestations and laboratory examinations when the disease reactivated were similar to those when newly diagnosed. Patients received individual treatments when the disease reactivated. Reactivation of the disease was common in about 6 weeks after initial treatments. Four patients survived and their mean survival time was 182.50±40.52 days. Eight patients died and the overall mortality rate was 67 %. The mean survival time of the death patient was 14.25 ± 7.09 days. Conclusion： Reactivated sHLH has a rapid progression and high mortality. We should conduct a comprehensive evaluation when the disease reactivates. It may be effective to increase the dosage of eorticoids.

关 键 词：继发性噬血细胞性淋巴组织细胞增多症 疾病再活动 临床分析 

分 类 号：R557[医药卫生—血液循环系统疾病]