Division of Hematology-Oncology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
Department of Health Sciences and Technology, Samsung Advanced Institute for Health Sciences and Technology, Sungkyunkwan University, Seoul, Korea.
Korean J Intern Med. 2020 Jul;35(4):946-956. doi: 10.3904/kjim.2018.396. Epub 2019 Nov 25.
BACKGROUND/AIMS: There are limited data about the influence of the central nervous system (CNS) involvement on the prognosis for patients with the Asian variant of intravascular large B-cell lymphoma (IVLBCL).
We analyzed 46 patients who were diagnosed with IVLBCL between 2001 and 2018. All patients were treated with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP).
CNS involvement was diagnosed by cerebrospinal fluid analysis (n = 6) and brain imaging (n = 5) in 11 patients at diagnosis, and four cases with CNS relapse were found. Thus, 15 patients had CNS involvement (15/46, 33%). The clinical characteristics were not different between patients with and without CNS involvement, but all patients with CNS involvement belonged to the high-risk group of CNS-International Prognostic Index (IPI). Thirty-one patients achieved a complete response (67%, 31/46) whereas eight patients showed disease progression and six patients died after the first or second cycle of R-CHOP. CNS-directed therapy such as high-dose methotrexate was combined with R-CHOP for patients with CNS involvement, and five patients were alive without relapse. The median overall survival of all patients was 45.0 months, and overall survival was not different according to the involvement of CNS.
The treatment outcome of patients with the Asian variant of IVLBCL is still not satisfactory. The prediction of CNS involvement based on the clinical features including CNS-IPI score might not serve to identify patients at high risk of CNS involvement, either. Thus, more effective strategies for diagnosis and treatment should be developed.
背景/目的：中枢神经系统（CNS）受累对亚洲型血管内大 B 细胞淋巴瘤（IVLBCL）患者预后的影响数据有限。
我们分析了 2001 年至 2018 年间诊断为 IVLBCL 的 46 例患者。所有患者均接受利妥昔单抗、环磷酰胺、多柔比星、长春新碱和泼尼松（R-CHOP）治疗。
11 例患者在诊断时通过脑脊液分析（n=6）和脑成像（n=5）诊断出 CNS 受累，4 例发生 CNS 复发，因此 15 例患者存在 CNS 受累（15/46，33%）。CNS 受累患者与无 CNS 受累患者的临床特征无差异，但所有 CNS 受累患者均属于 CNS-国际预后指数（CNS-IPI）的高危组。31 例患者达到完全缓解（67%，31/46），8 例患者出现疾病进展，6 例患者在 R-CHOP 第一或第二周期后死亡。对于 CNS 受累的患者，采用大剂量甲氨蝶呤联合 R-CHOP 进行 CNS 定向治疗，5 例患者无复发存活。所有患者的中位总生存期为 45.0 个月，CNS 受累与总生存期无关。
亚洲型 IVLBCL 患者的治疗效果仍不理想。基于包括 CNS-IPI 评分在内的临床特征预测 CNS 受累可能无法识别高危 CNS 受累患者。因此，应开发更有效的诊断和治疗策略。
