29例原发纵隔大B细胞淋巴瘤临床特征及长期随访结果

Clinical Characteristics and Long-term Follow-up of 29 Patients with Primary Mediastinal Large B Cell Lymphoma

本研究旨在探讨原发纵隔大B细胞淋巴瘤的临床特点、病理特征、治疗方法及预后因素。回顾性总结2000年1月至2013年11月我院收治的29例原发纵隔大B细胞淋巴瘤(PM LBCL)的临床资料并进行相关数据分析。结果表明,29例病理确诊为PMLBCL患者,中位年龄32岁,临床表现以纵隔巨块(72.4%)、上腔静脉综合征(51.7%)、呼吸困难(62.1%)、浆膜腔积液(48.3%)多见,其中62.1%存在结外侵犯,62.1%合并胸腔外受累。按照Ann-Arbor分期,16例(55.1%)为Ⅰ-Ⅱ期、13例(44.9%)为Ⅲ-Ⅳ期,12例(41.4%)合并B组症状。在本组29例患者中2例失访,中位随访29月,余27例患者中17例完全缓解,5例部分缓解,1例复发,4例死亡,死亡原因均为疾病进展,中位生存时间为29月,5年总生存率(OS)达85.2%,其中RCHOEP方案化疗组5年总生存率为94.4%,CHOEP方案化疗组5年总生存率为75%。8例行自体造血干细胞移植及1例行异基因造血干细胞移植患者随访至今均为持续CR。单因素分析显示,白蛋白和LDH水平升高,超过2倍正常上限,初治疗效、IPI评分具有预后参考意义,但多因素分析显示均非独立预后因素。结论:原发纵隔大B细胞淋巴瘤具有独特的临床特征,RCHOEP化疗方案初步效果肯定,值得进一步扩大病例探索。

This study was aimed to investigate the clinical manifestationpathological featurestreatment and related prognosis factors of primary mediastinal large B cell lymphoma（PMLBCL）.The clinical data of 29 PMLBCL patients admitted in Peking University First Hospital were summarized and the related factors were analyzed retrospectively from January 2000 to November 2013.The results showed that 29 patients with the median age 32 were all pathologically diagnosed as PMLBCL.The main clinical features included mediastinal bulk mass（ 72.4%）,superior vena caval syndrome（51.7%）,dyspnea（62.1%）,serous membrane fluid（48.3%）,with 62.1% extranodal invasion and 62.1%extra-thoracic involvement.According to Ann-Arbor stage16 patients（55.1%）were classified to stage Ⅰand 13patients44.9%to stage Ⅲ12 patients（41.4%）had B symptoms.Among the 29 patients2 patients failed to be followed and the others were followed for the median time of 29 months17 patients achieved CR5 patients achieved PR1 patient replased and 4 patients died of disease progression.The 5- year overall survival rate（OS）was 85.2% in which RCHOEP regimen group patients had OS 94.4% and CHOEP group patients had OS 75%8 patients underwent auto-HSCT and 1 patients underwent allo-HSCT who kept in CR state.Univariate anylasis by log-rank test showed albumin level and LDH2 ULNthe initial therapy response and IPI score were prognostic factors,but neither were independent prognostic factors by Cox Regression Model.It is concluded that PMLBCL has distinct clinical features.RCHOEP chemotherapy regimen can achieve satisfactory resultsbut needs to be explored by further clinical trials.