非霍奇金淋巴瘤相关性噬血细胞综合征32例临床特点和疗效分析

Clinical features and treatment outcome of non-Hodgkin’s lymphoma-associated hemophagocytic syndrome: report of 32 cases

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。

Abstract:

Objective To investigate the clinical features, treatment efficiency and prognosis of non-Hodgkin’s lymphoma-associated hemophagocytic syndrome (LAHS). Methods Clinical data of 32 LAHS patients treated in the Department of Hematology in Ruijin Hospital from January 2005 to December 2013 were retrospectively collected. Kaplan-Meier survival analysis was used to estimate survival functions. Cox regression model was performed to evaluate 14 factors affecting survival. 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 predominant pathological subtypes. Of the 23 patients who were treated with the regimens containing etoposide, 8 patients (35%) achieved complete remission (CR) and 6 patients (26%) partial remission (PR), with an overall 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 more efficient 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 CR non-achievement (P＝0.001) and subnormal hypofibrinogenemia (＜1.5g/L, P＝0.031) were the predictive parameters for total survival rate. Conclusion LAHS is usually accompanied with multi-organ dysfunctions, and has rapid progression and poor prognosis. Regimens containing etoposide significantly improve the treatment outcome of those patients.