非特异性外周T细胞淋巴瘤的临床特征与治疗结果分析

目的对非特异性外周T细胞淋巴瘤（PTCL—U）的临床特点、治疗和预后情况进行回顾分析,并探讨治疗方法的改进。方法收集1995年至2004年中国医学科学院肿瘤医院收治的有完整病例资料的78例PTCL—U,所有病例均按修订的欧美淋巴瘤分类（REAL分类）和WHO2001年分类进行病理分型,并对临床治疗和预后进行分析。结果确诊时中位年龄39（9～75）岁,男58例,女20例,临床分期Ⅰ／Ⅱ期占41％,Ⅲ／Ⅳ期占59％,结外受累占50．2％,乳酸脱氢酶（LDH）增高占38．0％,结外侵犯2处以上占29．5％,有B症状者占37．2％,国际预后指数（IPI）≥2分者62．8％;一线放化疗者34例,一线化疗者43例,单纯放疗者1例。首程治疗后完全缓解率为55．1％、部分缓解率为25．6％、进展＋稳定率为19．2％,13例患者在一线治疗缓解后进行了自体造血干细胞移植（ASCT）。存活患者中位随访58（1～116）个月。全组患者的5年总生存率和无进展总生存率为41．4％和33．8％。缓解后移植者的5年总生存率为61．5％（常规剂量治疗者为52．3％,P=0．894）。单因素分析显示预后不良因素包括Ⅲ／Ⅳ期病变、LDH增高、骨髓受侵、行为状态不佳。多因素分析显示LDH增高是独立的预后不良因素。淋巴瘤IPI和PTCL—U预后指数可以预测生存。结论PTCL—U发病时多为晚期、易侵犯结外,中危、高危患者的治疗效果不佳,一线治疗缓解后行ASCT安全可行,需要探讨新的一线治疗方案。

Clinical features and treatment outcomes of peripheral T-cell lymphoma, unspecified: a report of 78 cases

OBJECTIVE: To summarize the clinical features and treatment outcomes of peripheral T-cell lymphoma, unspecified (PTCL-US). METHODS: The medical records of 78 patients with PTCL-US classified according to the revised European and American lymphoma (REAL) or WHO criteria, 58 males and 20 females, aged 39 (9 - 75), 46 of them (59%) being at the stages III/VI and 40 cases (51.2%) with extranodal involvement), treated from Jan 1994 to Dec 2004 in the Cancer Hospital/Institute, Chinese Academy of Medical Sciences, were retrospectively analyzed. The patients were followed up for 58 months. RESULTS: Thirty patients (38%) were with high level lactate dehydrogenase (LDH). 34 cases (43. 7%) were treated with chemoradiotherapy, and 43 (55%) with chemotherapy alone. After the first line treatment the complete recovery (CR) rate and partial recovery (PR) rate were 55.1% (43/78) and 25.6% (20/78) respectively. 15 cases (19.2%) showed progressive disease (PD) or stable disease (SD). Achieving CR or PR after first-line treatment, 13 cases received autologous peripheral blood stem cell transplantation (APBSCT). The 5-year overall survival rate (OS) and 5-year progressive-free survival rate (PFS) were 41.4% and 33.8% respectively. The 5-year OS of the CR, PR, and PD/SD groups were 65.1%, 21.9%, and 0% respectively. The 5-year OS for the patients treated with first-line APBSCT was 61.5%, not significantly different from that of the patients treated with conventional dose therapy alone (52.3%, P = 0.894). Univariate analysis showed that the factors associated with a worse OS included stage III/IV (P = 0.001), high LDH (P = 0.0001), behavior state score >or= 2 (P = 0.001), and bone marrow involvement (P = 0.011). Multivariate analysis showed that LDH level was an independent factor predictive of survival (P = 0.001). International prognostic index and prognostic index for peripheral T-cell lymphoma both predicted the overall survival. CONCLUSION: A rare lymphoma with aggressive presentation, PTCL-US responds poorly to conventional treatment. The prognosis of the cases with high risk is bad. APBSCT is safe and feasible when CR or PR is achieved after first line treatment.