摘要
目的：探讨非霍奇金淋巴瘤相关性噬血细胞综合征（LAHS）的临床特点、治疗和预后。方法回顾性分析2005年1月至2013年12月上海交通大学附属瑞金医院血液科收治的32例LAHS患者的临床资料。用Kaplan-Meier法分析患者的总生存时间，应用Cox相关模型对可能影响生存的14项因素进行回归分析。结果32例患者中，国际预后指数（IPI）评分高危（4～5分）有7例（22%）。淋巴瘤病理分型以T细胞或自然杀伤细胞（NK）/T细胞型为主。32例患者中，有23例接受了含有依托泊苷的化疗方案，其中完全缓解（CR）8例（35%），部分缓解（PR）6例（26%），总有效率（ORR）达61%。有9例患者未接受联合依托泊苷的化疗方案，CR仅1例（11%），无患者达PR，两组比较，疗效有统计学差异（χ2＝4.874，P＝0.036）。本研究中，32例非霍奇金LAHS的患者中位生存时间为122d（95%CI：79.0～165.0），多因素分析结果显示未达CR（P＝0.001）和纤维蛋白原（Fg）低于正常（＜1.5g/L， P＝0.031）是影响患者总生存率的相关预后因素。结论 LAHS常伴有多个脏器受损的表现，病情进展迅速，预后差。含有依托泊苷的化疗方案，有助于改善患者的治疗效果。
ObjectiveTo investigate the clinical features, treatmentefficiency and prognosis of non-Hodgkin’s lymphoma-associated hemophagocytic syndrome （LAHS）.MethodsClinical data of 32 LAHS patients treated intheDepartment ofHematologyinRuijin Hospitalfrom January 2005 to December 2013were retrospectively collected. Kaplan-Meiersurvival analysiswas used to estimate survival functions. Cox regression modelwasperformedto evaluate 14 factors affectingsurvival. Results Among the 32 patients, 7（22%）were of high risk（4-5 points）according to the international prognostic index （IPI）. T-cell or natural killer （NK）/T cell subtypes were the most predominantpathologicalsubtypes. Of the 23 patients who were treated with the regimenscontainingetoposide, 8 patients （35%） achieved complete remission （CR） and 6 patients （26%） partial remission （PR）, withanoverall response rate （ORR）of 61%. Of the 9 patients who were treated with the regimens without etoposide, only 1 patient （11%） achieved CR and none PR （0%）. The regimens with etoposide were moreefficient than those without etoposide （Chi square=4.874,P=0.036）. The median overall survival （OS） of the cohort was 122 days （95%CI=79.0165.0）. Multivariate analysis showed that CRnon-achievement（P=0.001）and subnormal hypofibrinogenemia （〈1.5g/L,P=0.031） were the predictive parameters for total survival rate.ConclusionLAHS is usuallyaccompanied with multi-organ dysfunctions, and has rapid progression and poor prognosis. Regimens containing etoposide significantly improve the treatment outcome of those patients.
出处
《中华老年多器官疾病杂志》
2015年第11期817-821,共5页
Chinese Journal of Multiple Organ Diseases in the Elderly
关键词
非霍奇金淋巴瘤相关性噬血细胞综合征
治疗
预后
non-Hodgkin’s lymphoma-associated hemophagocytic syndrome
treatment
prognosis
