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Abstract：
The purpose of our study is to determine the outcome of patients with systemic non-Hodgkin lymphoma presenting with neurologic localization at diagnosis, as well as the impact of consolidation in terms of high-dose therapy followed by autologous stem cell transplantation. Newly diagnosed non-Hodgkin lymphoma patients with concomitant systemic and neurological involvement at diagnosis were included in this study. Sixty patients (37 males; 25 females) were included. Median age was 61 years (23-85 years). Histological subtype was mainly diffuse large B-cell lymphoma (n = 54; 90%). The International prognostic index was over 2 in 41 (72%) patients. Median number of extranodal sites was 2 (range: 1-5). Central nervous system involvement alone was documented in 48 patients. Paravertebral involvement with epidural mass and cord compression and positive cerebrospinal fluid were present in 7 patients. Five patients had both central nervous system and epidural involvement. First-line chemotherapy was mainly anthracycline-based (88%) plus high-dose methotrexate (74%) with or without cytarabine. Consolidation with high-dose therapy followed by autologous stem cell transplantation was performed in 19 patients. For the whole population, overall response rate after induction chemotherapy was 76%. Three-year progression-free survival and overall survival were 42 ± 7% and 44 ± 7%, respectively. For patients under 66 years of age, consolidation strategy using high-dose therapy followed by autologous stem cell transplantation positively impacted 3-year overall survival and progression free survival (P = 0.008) and (P = 0.003), respectively. In multivariate analysis, high-dose therapy had a positive impact on 3-year overall survival and progression-free survival for the whole population as well as for patients under 66 years old in CR after induction therapy (OS [HR=0.22 (0.07-0.67)] and progression-free survival [HR = 0.17 (0.05-0.54)]). In conclusion, non-Hodgkin lymphoma prognosis with concomitant systemic and neurological involvement at diagnosis is poor with a high risk of relapse when treated with conventional chemotherapies alone. This retrospective study supports the feasibility and the potential benefit of a consolidative strategy with high-dose therapy followed by autologous stem cell transplantation in this subset of patients. This strategy and the best intensive chemotherapy regimen remain to be validated in prospective trials.
摘要：
我们研究的目的是确定诊断时表现为神经定位的系统性非霍奇金淋巴瘤患者的预后。以及大剂量治疗后自体干细胞移植对巩固的影响。本研究包括新诊断的非霍奇金淋巴瘤患者，在诊断时伴有全身和神经系统受累。包括60例患者（男性37例；女性25例）。中位年龄为61岁（23-85岁）。组织学亚型主要为弥漫性大B细胞淋巴瘤（n=54；90％）。41例（72％）患者的国际预后指数超过2。结外位点的中位数为2(范围:1-5)。48例患者仅中枢神经系统受累。7例患者存在椎旁受累，硬膜外肿块，脊髓受压和脑脊液阳性。五名患者既有中枢神经系统又有硬膜外受累。一线化疗主要是蒽环类药物（88％）加上大剂量甲氨蝶呤（74％），有或没有阿糖胞苷。在19例患者中进行了大剂量治疗合并，然后进行自体干细胞移植。对于整个人口来说，诱导化疗后的总缓解率为76%.三年无进展生存率和总生存率分别为42±7%和44±7%。分别。对于66岁以下的患者，使用大剂量治疗后自体干细胞移植的巩固策略对3年总生存期和无进展生存期(P=0.008)和(P=0.003)产生积极影响,分别。在多变量分析中，大剂量治疗对整个人群以及66岁以下患者的3年总生存期和无进展生存期有积极影响,患者在诱导治疗后的CR(OS[HR=0.22(0.07~0.67)]和无进展生存期[HR=0.17(0.05~0.54)]).总之,非霍奇金淋巴瘤在诊断时伴有全身和神经系统受累的预后较差,单独使用常规化疗治疗时复发风险较高.这项回顾性研究支持在这一子集患者中采用大剂量治疗后进行自体干细胞移植的巩固策略的可行性和潜在益处。这种策略和最佳的强化化疗方案仍有待在前瞻性试验中验证。
