Kuitunen Hanne, Tokola Susanna, Siniluoto Topi, Isokangas Matti, Sonkajärvi Eila, Alahuhta Seppo, Turpeenniemi-Hujanen Taina, Jantunen Esa, Nousiainen Tapio, Vasala Kaija, Kuittinen Outi
Department of Oncology and Radiotherapy, Oulu University Hospital, Oulu University, Kajaanintie 50, P.O. Box 5000, 90014, Oulu, Finland.
Department of Radiology, Oulu University Hospital, Oulu University, Oulu, Finland.
J Neurooncol. 2017 Jan;131(2):293-300. doi: 10.1007/s11060-016-2293-8. Epub 2016 Oct 17.
Primary central nervous system lymphoma (PCNSL) is a rare brain tumour with a dismal prognosis. Several phase II studies with high-dose methotrexate-based regimens have shown promising early results, but in all hospital-based data published so far, the disease outcome has been poor. Patients with relapsed or refractory disease have a dismal prognosis. We performed retrospective analysis to evaluate results and tolerabilities of BBBD therapy in combination with high-dose therapy supported by autologous stem cell transplantation. We analysed 25 patients (age range: 40-71 years) who were treated in first or second line with BBBD therapy. When we started BBBD treatment, patients had relapsed or refractory PCNSL or they did not tolerate Bonn-like therapy. In recent years, some of the patients were treated in first line. We found promising response rates. Altogether 19 (76 %) of the patients achieved a complete response (CR). Two-year progression-free survival (PFS) and overall survival (OS) rates were 61 and 57 % respectively and the five-year OS was 47 %. Patients who were treated with a five-drug therapy had a very promising prognosis. The CR rate was 100 % in first-line therapy and 60 % in relapsed cases. These findings suggest that BBBD is a promising therapy for PCNSL, especially for patients in first line, but also for patients with relapsed or refractory disease after conventional chemotherapy, who commonly have a very poor prognosis. Treatment-related toxicity was generally manageable. Thus, BBBD followed by ASCT could be a treatment of choice in transplant-eligible patients with PCNSL.
原发性中枢神经系统淋巴瘤（PCNSL）是一种预后不佳的罕见脑肿瘤。几项采用基于大剂量甲氨蝶呤方案的II期研究已显示出有希望的早期结果，但在迄今为止发表的所有基于医院的数据中，疾病结局都很差。复发或难治性疾病患者的预后不佳。我们进行了回顾性分析，以评估血脑屏障破坏（BBBD）疗法联合自体干细胞移植支持的大剂量疗法的效果和耐受性。我们分析了25例（年龄范围：40 - 71岁）接受一线或二线BBBD治疗的患者。当我们开始BBBD治疗时，患者患有复发或难治性PCNSL，或者他们不耐受类似波恩方案的治疗。近年来，一些患者接受了一线治疗。我们发现了有希望的缓解率。总共19例（76%）患者实现了完全缓解（CR）。两年无进展生存期（PFS）和总生存期（OS）率分别为61%和57%，五年OS为47%。接受五药疗法治疗的患者预后非常有希望。一线治疗的CR率为100%，复发病例为60%。这些发现表明，BBBD是一种有希望的PCNSL治疗方法，特别是对于一线患者，对于常规化疗后复发或难治性疾病的患者也适用，这些患者通常预后很差。与治疗相关的毒性一般是可控的。因此，BBBD联合自体干细胞移植可能是适合移植的PCNSL患者的一种治疗选择。
