原发性中枢神经系统淋巴瘤诊治进展

原发性中枢神经系统淋巴瘤(primarycentralnervoussystemlymphoma,PCNSL)是原发于脑、眼和脊髓的非霍奇金氏淋巴瘤,临床上较罕见,近数十年来发病率逐渐上升。PCNSL多见于老年患者,病理类型、预后因素和治疗方案有别于全身性非霍奇金淋巴瘤(NHL)。PCNSL病理类型以弥漫大B细胞为主,年龄和(performancestatus)PS是最重要的预后因素,预后较全身性NHL差。目前PCNSL尚无标准治疗方法,一般采用化疗和放疗联合的治疗措施。手术仅起到诊断作用。PCNSL对放疗高度敏感,但单纯放疗有效维持时间短。化疗在治疗PCNSL中不可缺少,但CHOP方案对PCNSL无效。大剂量甲氨蝶呤(HD-MTX)是最有效的药物,大剂量阿糖胞苷(HD-AraC)是常用的药物之一。因此,现阶段PCNSL采用含HD-MTX或/和HD-AraC等的联合化疗,同时鞘内注射MTX、AraC和地塞米松(DXM)。放疗疗效差,放疗应在化疗结束后进行。联合化、放疗对60岁以上患者可造成严重的远期神经毒性,对老年患者可选择单纯化疗和延迟放疗的治疗方法。自体造血干细胞支持下超大剂量化疗疗效优于历史对照。新药Temozolomide、Temozolomide联合美罗华、Topotecan、放射免疫治疗药物Y90标记CD20单抗等对PCNSL取得一定效果,值得进一步研究。

Recent Advance on Primary Central Nervous System Lymphoma

The primary central nervous system lymphoma ( PCNSL ) is a rare subtype of NHL confined to brain, spinal cord or eye. The incidence steadily increases during the past decades. The pathologic, prognosis and therapeutic pattern of PCNSL is different from systemic NHL. No standard treatment is available so far. Surgery is performed only for diagnosis. Chemotherapy is indispensable for the treatment of PCNSL, but CHOP failed to prolong the survival. High-dose methotrexate is the most effective drug. PCNSL is sensitive to irradiation, but responses to radiotherapy alone are short-lived. MTX-based chemotherapy followed by whole-brain radiotherapy prolonged survival but is associated with delayed neurotoxicity especially in patients older than 60 years. An new approach is attempted to treat PCNSL with chemotherapy alone with defered radiotherapy in old patients with similar survival rencently. Age, performance status and use of HD-MTX containing regimen are the most important prognostic factors. Autologous stem cell transplantation yielded promising results for recurrent PCNSL. Clinical investigation with Temozolomide, Temozolomide plus rituximab, Topotecan or Zevalin have been reported. Further studies with these new drugs are warranted.