基于单中心的血管免疫母细胞性T细胞淋巴瘤的临床特征及预后分析

Clinical characteristics and prognosis of angioimmunoblastic T-cell lymphoma based on single clinical center

目的:探讨血管免疫母细胞性T细胞淋巴瘤(angioimmunoblastic T-cell lymphoma, AITL)的临床特征及预后。方法:回顾性分析2005年11月至2017年6月复旦大学附属中山医院血液科收治的36例经病理及免疫组织化学确诊为AITL患者的临床资料。采用log-rank生存分析及Cox多因素分析法分析影响患者总生存期(overall survival, OS)的预后因素。结果:36例患者中位年龄62(36～79)岁,男性22例(61.1%),Ann Arbor分期Ⅲ/Ⅳ期35例(97.2%),有B症状(发热、盗汗、体质量减轻)25例(69.4%)。国际预后指数(international prognostic index, IPI)评分0～1分3例(8.3%),2分5例(13.9%),3分13例(36.1%),4、5分共15例(41.7%)。患者中位生存时间为24.4个月,3年OS率为49.2%。5例患者接受西达本胺治疗,1例为完全缓解(CR)后复发,经GemOx方案联合西达本胺治疗,再次获得CR;另4例均为一线治疗过程中出现疾病进展(PD),二线治疗的同时联合西达本胺口服,其中1例出现疾病稳定(SD),3例死亡。单因素生存分析结果显示,多浆膜腔积液(P=0.05)、C反应蛋白(CRP)>40 mg/L(P=0.001)、中性粒细胞升高(P=0.004)是影响患者OS的不良因素。多因素分析结果显示,CRP>40 mg/L[(HR=9.185,95%置信区间(CI) 2.599～32.462,P=0.001]、血小板(PLT)<150×10~9/L(HR=3.851,95%CI 1.328～11.167,P=0.013)、多浆膜腔积液(HR=3.054,95%CI 1.105～8.438,P=0.031)是影响患者OS的独立不良预后因素。结论:AITL患者预后差,CRP>40 mg/L、PLT<150×10~9/L、多浆膜腔积液是其独立风险因素。

Objective:To explore the clinical characteristics and prognostic factors of patients with angioimmunoblastic T-cell lymphoma (AITL).Methods: The clinical characteristics and prognostic factors of 36 patients with AITL were retrospectively analyzed, all of whom were treated at the Department of Hematology, Zhongshan Hospital, Fudan University between November 2005 and June 2017, and were confirmed by pathology and immunohistochemistry. Log-rank survival analysis and Cox multivariate analysis were used to analyze the prognostic factors affecting the overall survival (OS) of patients. Results: The median age of 36 patients was 62 (36-79) years old, with 61.1% males ( n =22), 97.2% Ann Arbor stage Ⅲ/Ⅳ( n =35), and 69.4% with B symptoms ( n =25). Three cases (8.3%) were categorized as 0-1, 5 cases (13.9%) as 2, 13 cases (36.1%) as 3, and 15 cases (41.7%) as 4 and 5 for the international prognostic index (IPI). The median survival time was 24.4 months, and the 3-year OS rate was 49.2%. Five cases were treated with chidamide, one of whom had relapse after complete remission (CR), after GemOx combined with chidamide treatment, reached CR again. The other 4 cases had disease progression (PD) during the first-line treatment. They were treated with chidamide as a part of the second-line treatment. Among whom, 1 case had stable disease (SD) and 3 cases died. Univariate survival analysis showed that multiple serous effusion ( P =0.05), CRP >40 mg/L ( P =0.001), and neutrophil >7×10 9/L ( P =0.004) were the adverse prognostic factors affecting OS. Multivariate analysis showed that CRP >40 mg/L (HR=9.185, 95% CI 2.599-32.462, P =0.001), PLT <150×10 9/L (HR=3.851, 95% CI 1.328-11.167, P =0.013), multiple serous effusion (HR= 3.054, 95% CI 1.105-8.438, P =0.031) were independent prognostic factors for OS. Conclusions: The prognosis of AITL is poor. CRP>40 mg/L, PLT<150×10 9/L, and multiple serous effusion might be independent adverse prognostic factors for OS.