原发性中枢神经系统淋巴瘤临床治疗结果分析

Clinical Outcomes of Primary Central Nervous System Lymphoma (PCNSL): Clinical Experience of Single Center

原发性中枢神经系统淋巴瘤(PCNSL)是一种罕见的淋巴瘤,近数十年来发病率逐渐上升。由于部位特殊,现有淋巴瘤的预后因素和常规治疗方案不适用于PCNSL。PCNSL发病率低,目前尚无标准的预后因素和一线标准治疗方案,但随着临床治疗经验的不断积累,在治疗上已取得一定的共识,含大剂量MTX的全身化疗结合放疗使PCNSL中位生存期有较明显提高。本研究目的是了解PCNSL的临床特征和治疗结果,评价VM26/Ara-C/HD-MTX方案加放疗治疗PCNSL的疗效与毒性,寻求有效简单的治疗方案。方法:回顾性分析1990～2003年我院13例PCNSL的临床资料。其中采用VM26/Ara-C/HD-MTX加鞘注方案治疗5例PC.NSL。结果:13例PCNSL,男性6例,女性7例,中位年龄47岁。病理类型83%为弥漫性B细胞性。1例单纯手术切除,12例综合治疗。中位随访15个月,死亡5例,尚未达到中位生存期。5例以VM26+HD-Ara-C+HD-MTX加鞘内注射MTX+Ara-C+DXM化疗,随后加全脑放疗,有效率100%,完全缓解(CR)率80%。Ⅲ-Ⅳ度血液学毒性占30%,感染占3%,未发现明显的神经毒性。结论:手术在PCNSL中起诊断作用,术后加化/放疗等综合治疗可延长生存期,VM26+HD-Ara-C+HD-MTX方案近期疗效高,毒性可以接受,值得进一步研究。

BACKGROUND & OBJECTIVES: Primary central nervous system lymphoma (PCNSL) is a rare type of lymphoma. The incidence steadily increases during the past decades. The international prognostic index (IPI)and standard treatment of systemic NHL are not suitable for PCNSL. Although these were still no standard treatment and prognostic factors for PCNSL so far, widely accepted therapeutic modality has been established based on accumulation of clinical experience. The survival of PCNSL patients has been improved dramatically by high-dose methotrexate contain-ing regimen combined with radiotherapy in recent years. The purpose of our study is to evaluate clinical characteristic and prognosis of PCNSL, the efficacy and toxicity of systemic chemotherapy especially the protocol combining systemic VM26/Ara-C/HD-MTX and intrathecal chemotherapy in treatment of PCNSL so as to identify a simple and effective therapeutic protocol. METHODS: Thirteen cases of PCNSL patients treated from 1991 to 2003 were analyzed retrospective-ly. Five patients were treated with VM26/Ara-C/HD-MTX regimen. RESULTS: There were 6 male and 7 female with median age of 46 years old. Eighty-three percent of all cases were classified as diffuse large B cell lymphoma. All except one patient were treated by multimodality and reminding one was treated with surgery alone. The median follow- up was 15 months and more than half are still surviv-ing. Four patients died due to recurrence of the dis-ease and another one case died of severe infection caused by grade 4 neutropenia. Five patients were treated with 4-6 courses of VM26/Ara-C/ HD-MTX with intralhecal MTX+Ara C+DXM chemotherapy followed by whole brain irradiation plus involved field boost. The overall response for this regimen is 100% with 80% clinical remission (CR) rate. The toxicity is mild. No neurotoxicity was found. CONCLUSION: Chemothera-py combined with radiotherapy play an important role in the management of PCNSL. Surgery was merely diagnostic. The preliminary result of our clinical trial showed response rate to VM26/Ara -C/HD-MTX is high and the toxicity is accept-able. Further investigation is warranted.