Division of Hematology-Oncology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, 50 Irwon-dong, Gangnam-gu, Seoul, 135-710, South Korea.
Ann Hematol. 2011 Dec;90(12):1391-8. doi: 10.1007/s00277-011-1225-0. Epub 2011 Apr 9.
Diffuse large B-cell lymphoma (DLBCL) constitutes most primary central nervous system (CNS) lymphoma (PCNSL), whereas T-cell, low-grade and Burkitt's lymphomas (BL) are rarely encountered. Due to the paucity of cases, little is known about the clinical features and treatment outcomes of PCNSL other than DLBCL. The objective of this study was to describe the clinical characteristics and outcomes for patients with PCNSL other than DLBCL. Fifteen patients, newly diagnosed with PCNSLs other than DLBCL between 2000 and 2010, were included. The male to female ratio was 0.67:1 with a median age of diagnosis of 31 years (range 18-59). Pathologic distributions were as follows: peripheral T-cell lymphoma (PTCL; n=7), marginal zone B-cell lymphoma (MZBCL; n=1), lymphoplasmacytic lymphoma (LPL; n=2), Burkitt's lymphoma (n=1), other unspecified (T-cell lineage, n=2; B-cell lineage, n=2). Thirteen patients (87%) showed Eastern Cooperative Oncology Group performance score (ECOG PS) 1-2. The remaining two were one PTCL patient and one Burkitt's lymphoma patient. Of the nine patients with T-cell lymphoma, five (56%) had multifocal lesions, and one (20%) with LPL of the five patients with B-cell lymphoma showed a single lesion. Leptomeningeal lymphomatosis was identified in two patients (one with Burkitt's lymphoma and one with unspecified B-cell lymphoma). Two patients (22%) with T-cell lymphoma died 7.7 and 23.3 months later, respectively, due to disease progression, despite HD-MTX-based therapy. Six patients with T-cell lymphoma (6/9, 66.7%) and four patients with low-grade B-cell lymphoma (4/5, 80%) achieved complete response and have survived without relapse (Table 3). One patient with Burkitt's lymphoma showed poor clinical features with ECOG PS 3, deep structure, multifocal, and leptomeningeal lymphomatosis, and died 7.6 months after the initiation of treatment. In comparison with previously reported DLBCLs (median OS 6.4 years, 95% CI 3.7-9.1 years), T-cell lymphoma showed equivocal or favorable clinical outcomes and low-grade B-cell lymphomas, such as MZBCL and LPL, had a good prognosis. However, primary CNS Burkitt's lymphoma presented poor clinical outcomes and showed a comparatively aggressive clinical course. In conclusion, primary CNS lymphoma other than DLBCL occurred more in younger patients and showed a generally good prognosis, except for Burkitt's lymphoma. Further research on treatment strategies for Burkitt's lymphoma is needed.
弥漫性大 B 细胞淋巴瘤（DLBCL）构成了大多数原发性中枢神经系统（CNS）淋巴瘤（PCNSL），而 T 细胞、低级别和伯基特淋巴瘤（BL）则很少见。由于病例数量较少，对于除 DLBCL 以外的 PCNSL 的临床特征和治疗结果知之甚少。本研究的目的是描述除 DLBCL 以外的 PCNSL 患者的临床特征和结局。2000 年至 2010 年间，共纳入 15 例新诊断为除 DLBCL 以外的 PCNSL 患者。男女比例为 0.67:1，中位诊断年龄为 31 岁（18-59 岁）。病理分布如下：外周 T 细胞淋巴瘤（PTCL；n=7）、边缘区 B 细胞淋巴瘤（MZBCL；n=1）、淋巴浆细胞淋巴瘤（LPL；n=2）、伯基特淋巴瘤（n=1）、其他未特指（T 细胞谱系，n=2；B 细胞谱系，n=2）。13 名患者（87%）的东部合作肿瘤学组表现评分（ECOG PS）为 1-2。其余两名患者分别为 1 名 PTCL 患者和 1 名伯基特淋巴瘤患者。在 9 名 T 细胞淋巴瘤患者中，有 5 名（56%）有多发病灶，在 5 名 B 细胞淋巴瘤患者中有 1 名（20%）LPL 表现为单发病灶。两名患者（一名伯基特淋巴瘤患者和一名未特指 B 细胞淋巴瘤患者）存在软脑膜淋巴瘤。两名 T 细胞淋巴瘤患者（22%）分别在 7.7 和 23.3 个月后因疾病进展而死亡，尽管接受了 HD-MTX 为基础的治疗。6 名 T 细胞淋巴瘤患者（6/9，66.7%）和 4 名低级别 B 细胞淋巴瘤患者（4/5，80%）达到完全缓解，并且没有复发（表 3）。一名患有伯基特淋巴瘤的患者表现出不良的临床特征，ECOG PS 为 3，累及深部结构，多发病灶，并且伴有软脑膜淋巴瘤，在治疗开始后 7.6 个月死亡。与先前报道的 DLBCL 相比（中位 OS 6.4 年，95%CI 3.7-9.1 年），T 细胞淋巴瘤的临床结局或可比较或较好，低级别 B 细胞淋巴瘤，如 MZBCL 和 LPL，预后良好。然而，原发性中枢神经系统伯基特淋巴瘤的临床结局较差，表现出侵袭性更强的临床病程。总之，除伯基特淋巴瘤外，原发性中枢神经系统淋巴瘤多见于年轻患者，预后总体良好。需要进一步研究伯基特淋巴瘤的治疗策略。
