原发性中枢神经系统淋巴瘤的治疗进展

原发性中枢神经系统淋巴瘤（primary central nervous system lymphoma ,PCNSL ）是一种罕见的结外淋巴瘤,其病理类型多为弥漫性大B 细胞淋巴瘤,影像学多表现为单发的脑实质深部病变,MRI 可有多种强化形态。目前,以大剂量甲氨蝶呤为主的化疗已成为PCNSL 的一线治疗。放、化疗的结合可延长患者的生存期,但神经毒性发生率较高。大剂量化疗联合自体干细胞移植对复发/ 难治性PCNSL 有效。替莫唑胺和利妥昔单抗不良反应小、耐受性好,可作为PCNSL 治疗的选择。PCNSL 的预后受血清LDH浓度、年龄、ECOG 评分/KPS 评分、脑脊液蛋白浓度、肿瘤定位等多种因素的影响。      

原发中枢神经系统淋巴瘤治疗进展

 【摘要】　原发性中枢神经系统淋巴瘤（PCNSL）是原发于脑、眼和脊髓的结外淋巴瘤,发病率低,老年患者多见,因其在庇护所内恶性侵袭性生长,使得治疗困难,预后不良。目前PCNSL尚无标准治疗方法,有效的治疗一般采用化疗和放疗联合的措施。手术不能完全切除病灶,仅起到诊断作用。大剂量甲氨蝶呤（HD-MTX）是最有效的药物,大剂量阿糖胞苷是常用的药物之一。PCNSL对放疗高度敏感,但单纯放疗有效维持时间短,对60岁以上患者可导致严重的远期神经毒性,影响预后,故主张延迟放疗。大剂量化疗联合自体干细胞移植通过提高剂量化疗可以克服肿瘤细胞耐药,提高药物生物利用度,替代全脑放疗,减少神经毒性。新药替莫唑胺、利妥昔单抗等对PCNSL取得一定效果,值得进一步研究。     

Temozolomide and methotrexate for primary central nervous system lymphoma in the elderly

Background Treatment for primary CNS lymphoma (PCNSL) in the elderly is associated with lower response rates and higher risks of acute and late delayed toxicity as compared to younger patients. Temozolomide has emerged as a new alternative treatment for PCNSL and constitutes an attractive option for the elderly because of its favorable toxicity profile. In this study we report outcomes of a consecutive series of PCNSL elderly patients initially treated with an innovative regimen combining methotrexate and temozolomide without radiotherapy or intra-thecal chemotherapy. Methods Histologically confirmed newly-diagnosed PCNSL patients older than 60 years were included. An induction chemotherapy was initially given (methotrexate 3 g /m² on days 1, 10, and 20, and temozolomide 100 mg/m² on days 1–5). Patients achieving a partial or complete response proceeded to a maintenance phase (up to 5 monthly cycles of methotrexate 3 g/m² on day 1, and temozolomide 100 mg/m² days 1–5). Non-responders were treated on an individual basis. Results Among the 23 included patients, a complete response was observed in 55%, and disease progressed in the other 45%. Median event-free survival was 8 months, and median overall survival was 35 months. Grades 3 or 4 toxicities included nephrotoxicity in three patients, and hematotoxicity in five; no neurotoxicity has been observed to date. One patient died while on treatment from complications of intestinal obstruction. Conclusion Our efficacy results are comparable to other reported regimens, with the advantages of a favorable toxicity profile, and absence of intra-thecal chemotherapy. Prospective, controlled studies are warranted to confirm such results.     

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。     

原发性中枢神经系统淋巴瘤的治疗进展

原发性中枢神经系统淋巴瘤（PCNSL）是一种少见疾病,至今尚无标准治疗方案。单纯放疗复发率高,生存期短。放疗宜在化疗结束后进行。化疗联合标准放疗明显降低了复发率,并延长了生存期,但神经毒性发生率高。老年患者易出现神经毒性,不建议放疗,应首选单纯化疗;年轻患者可将放疗作为难治复发时的二线治疗。目前,以大剂量甲氨蝶呤为主的化疗已成为PCNSL的一线治疗,大剂量阿糖胞苷为最常联合的药物。年轻患者可选用包含一些新药如甲基苄肼、替莫唑胺的化疗方案。替莫唑胺为老年患者一种有前途的新药。预防性鞘内化疗的必要性尚未达成共识。自体造血干细胞支持下的大剂量化疗对年轻的初发及复发PCNSL患者均有效。手术通常用于PCNSL诊断。糖皮质激素不宜在取得病理组织前使用。      

Primäre Zentralnervensystemlymphome (PZNSL)

Primary central nervous system lymphoma (PCNSL) comprises about 2?% of intracranial tumors. The vast majority of these tumors are diffuse large B-cell lymphomas; however, PCNSL differs from nodal lymphoma with a similar histopathology by its strong affinity for the CNS with infrequent systemic involvement, by its usually aggressive course and unusually high sensitivity to high-dose methotrexate (HDMTX). Thus, HDMTX represents the backbone of current therapy although the optimal treatment protocol for PCNSL has not yet been established due to the paucity of occurrence. Age and general condition of the patient are the most important prognostic factors. In younger patients curing is the goal and can probably be achieved in a fraction of patients by application of intensified chemotherapy protocols. In the elderly, however, a cure is mostly not possible and therapy optimization is still urgently needed. To spare patients with PCNSL the potentially devastating consequences of delayed treatment-related CNS toxicity is an important goal.     

Platine and cytarabine-based salvage treatment for primary central nervous system lymphoma

The aim of this study was to evaluate the efficacy and toxicity of two chemotherapy regimens based on platinum and cytarabine in association with etoposide and methylprednisolone (ESHAP) or with dexamethasone (DHAP) with or without Rituximab (± R) in patients with refractory or a relapsed Primary Central Nervous System Lymphoma (PCNSL). All consecutive patients from two French centers with refractory or relapsed PCNSL treated with ESHAP/DHAP ± R were included. We analyzed the overall response rate (ORR), toxicity and overall survival (OS) after salvage chemotherapy. Intensive chemotherapy and hematopoietic stem cell rescue (IC + HCR) was offered to patients less than 65 years of age and consisted of high-dose thiotepa, busulfan and cyclophosphamide. These results were compared with two previously reported series of PCNSL patients treated with the CYVE (high-dose cytarabine and etoposide) regimen at relapse. Twenty-two patients received a total of 60 DHAP/ESHAP cycles (median 3; range 1–5). The median age was 59 years. The ORR after salvage chemotherapy was 59%. Toxicity was mainly hematological, 18% of patients showing febrile neutropenia. There was no treatment-related death. ESHAP or DHAP regimens led to similar ORRs compared to the CYVE regimen in relapsed or refractory PCNSL, although they seemed less toxic. The therapeutic results of the ESHAP/DHAP regimens in relapsed or refractory PCNSL were also similar to those for relapsed systemic non-Hodgkin’s lymphomas (sNHL). Both chemotherapies, CYVE regimen and ESHAP/DHAP are treatment options to be considered in relapsed or refractory PCNSL, especially when IC + HCR is planned as a consolidation treatment. More efforts are still needed to improve the ORR at relapse.     

Salvage chemotherapy with temozolomide in primary CNS lymphomas: preliminary results of a phase II trial

Temozolomide is a well-tolerated alkylating agent, that is able to permeate the blood-brain barrier (BBB), and has additive cytotoxicity when given with radiotherapy (RT). A phase II trial assessing temozolomide 150 mg/m(2)/day, for 5 days every 28 days in primary central nervous system (CNS) lymphoma (PCNSL) patients with negative human immunodeficiency virus (HIV) serology, Eastern Cooperative Oncology Group (ECOG) performance status (PS)<4, previously treated with high-dose methotrexate-containing (HD-MTX) chemotherapy and/or RT was started. Twenty-three patients were enrolled. Median age was 60 years. Five complete remissions (median duration 6+ months; range 2-36 months), one partial response, four stable disease (median duration 7.2 months, range 2-16.5 months), and 13 progressions were observed. No major toxicities were observed, apart grade 3 vomiting in a single cycle. Main grade 1-2 toxicities were: 15% nausea, 6% vomiting, 9% fatigue and 9% neurological symptoms. This is the first prospective trial assessing single-agent activity in PCNSL at failure. Although some patients had a poor PS and had been heavily pre-treated, temozolomide yielded 26% objective responses and was well tolerated without any major toxicity.     

O6-methylguanine-DNA methyltransferase (MGMT) immunohistochemistry as a predictor of resistance to temozolomide in primary CNS lymphoma

Temozolomide, an alkylating agent, has shown promise in treating primary central nervous system lymphoma (PCNSL). The enzyme O⁶-methylguanine-DNA methyltransferase (MGMT) repairs alkylating damage, such as that induced by temozolomide. We hypothesized that MGMT immunohistochemistry would predict resistance to temozolomide in PCNSL. A retrospective study of newly-diagnosed and recurrent PCNSL patients treated at our institution was conducted to study the predictive value of MGMT immunohistochemistry for response to temozolomide. 20 patients who were treated with temozolomide as a single agent were identified during the study time period. 6/20 patients demonstrated a response, corresponding to an objective response rate of 30 % (95 % CI 8–52). Five patients with low MGMT level (<30 %) showed a response to temozolomide. Only one of 10 patients (10 %) with high MGMT level (≥30 %) exhibited a response to temozolomide. Small sample numbers precluded formal statistical comparisons. Two patients with complete response remain alive without progressive disease 6.7 and 7.2 years after temozolomide initiation. Immunohistochemistry can be performed on small biopsies to selectively assess MGMT status in tumor versus surrounding inflammation. MGMT analysis by immunohistochemistry may predict response to temozolomide in PCNSL and should be prospectively investigated.     

Current status and future of relapsed primary central nervous system lymphoma (PCNSL)

The treatment of primary central nervous system lymphoma (PCNSL) has centered around high-dose methotrexate and radiotherapy (RT). Methotrexate administered intra-arterially (IA) with blood-brain barrier disruption (BBBD) and without RT, has been a highly effective treatment with a 5 year survival of 42% without cognitive loss. The purpose of this analysis is to determine responses for patients with relapsed PCNSL treated with second line IA carboplatin-based chemotherapy with BBBD. Between February 1991 and April 2000, 37 relapsed PCNSL patients, most who failed front line therapy with methotrexate based chemotherapy, were treated at Oregon Health & Science University (OHSU) and Hadassah Hebrew University Hospital (HHUH) with IA carboplatin-based chemotherapy with BBBD. Nine patients had prior RT. The mean age was 57.5 years, and all but 1 patient were treated within 8 months after relapse. The median time for survival from first IA carboplatin/BBBD treatment was 6.8 months;however, 7 out of 37 patients survived > or = 27 months. Nine patients had radiographic complete response (CR), 4 patients had radiographic partial response (PR), 12 had stable disease (SD), 10 had progressive disease (PD), and 2 were non-evaluable. The median time to failure for patients with CR and PR was 9.1 months. One long-term survivor is alive at 91.0 months from first carboplatin/BBBD treatment. In conclusion, we show that relapsed PCNSL has shown sensitivity to second line IA carboplatin-based chemotherapy with BBBD. We have developed a new protocol using i.v. rituximab prior to BBBD with IA carboplatin, i.v. cyclophosphamide and i.v. etoposide phosphate. The long-term program goal is to consolidate dose-intensive chemotherapy with monoclonal antibody directed radiation. Because patients with recurrent PCNSL commonly continue to relapse even after obtaining a complete response to enhanced chemotherapy treatment, patients w ho complete or fail the above carboplatin/BBBD treatment regimen will be offered consolidation with radioimmunotherapy using zevalin (Ibritumomab tiuxetan), IDEC-2B8 conjugated with yttrium-90 (90Y).     

Temozolomide-induced partial response in a patient with primary diffuse leptomeningeal gliomatosis

Herein we describe the case of a patient with primary diffuse leptomeningeal gliomatosis (PDLG). After surgery and ventriculoperitoneal shunt placement, due to poor general conditions, the patient was not eligible for radiotherapy. For this reason we decided to start a systemic chemotherapy treatment with Temozolomide (150 mg/m² per day for 5 days every 4 weeks). After three cycles a partial response was achieved with a clear improvement of general conditions. In our knowledge, this is the first time that PDLG treatment with Temozolomide has been described.     

Second-line chemotherapy with temozolomide in recurrent oligodendroglioma after PCV (procarbazine, lomustine and vincristine) chemotherapy: EORTC Brain Tumor Group phase II study 26972.

BACKGROUND: Oligodendroglial tumors are chemosensitive, with two-thirds of patients responding to PCV combination chemotherapy with procarbazine, lomustine (CCNU) and vincristine. Temozolomide (TMZ), a new alkylating and methylating agent has shown high response rates in recurrent anaplastic astrocytoma. We investigated this drug in recurrent oligodendroglial tumors (OD) and mixed oligoastrocytomas (OA) after prior PCV chemotherapy and radiation therapy. PATIENTS AND METHODS: In a prospective non-randomized multicenter phase II trial patients were treated with TMZ 150 mg/m(2) on days 1-5 in cycles of 28 days for 12 cycles. Eligible patients had a recurrence after prior PCV chemotherapy, with measurable and enhancing disease as shown by magnetic resonance imaging. Pathology and all responses were centrally reviewed. RESULTS: Thirty-two eligible patients were included. In four patients the pathology review did not confirm the presence of an OD or OA. Twelve of 24 patients [50%, 95% confidence interval (CI) 29% to 71%] evaluable for response to first-line PCV chemotherapy had responded to PCV. Temozolomide was in general well tolerated; the most frequent side-effects were hematological. One patient discontinued treatment due to toxicity. In seven of 28 patients (25%, 95% CI 11% to 45%) with histologically confirmed OD an objective response to TMZ was observed. Median time to progression for responding patients was 8.0 months. After 6 and 12 months from the start of treatment, 29% and 11% of patients, respectively, were still free from progression. CONCLUSIONS: TMZ may be regarded as the preferred second-line treatment in OD after failure of PCV chemotherapy. Further studies on TMZ in OD are indicated.     

Primary central nervous system lymphoma: biological aspects and controversies in management

INTRODUCTION: This review was produced from the workshop on primary central nervous system lymphoma (PCNSL) at the European Cancer Conference (ECCO 13) in Paris in 2005. It covers the presentation and biological features of the disease (Professor Khe Hoang-Xuan). The role of chemotherapy, including the management of intraocular lymphoma and the use of high dose chemotherapy followed by autologous stem cell transplantation for PCNSL, is discussed (Dr. Andres Ferreri) as well as controversies in the use of whole brain radiotherapy (WBRT) after chemotherapy (Dr. Michele Reni). The topics covered with discussants at the workshop are also summarised. CONCLUSION: The imaging of the brain and the histopathology including detailed immunohistochemistry is of vital importance in making an accurate diagnosis of the disease and understanding the extent of spread of the disease in the CNS. The importance of high dose methotrexate (HDMTX; dose > or = 1g/m(2)), as the most active drug in the treatment of PCNSL, is stressed. The authors recommend that HDMTX alone or in combination with other active chemotherapy agents should be used to treat PCNSL followed by whole brain radiotherapy (WBRT) unless contraindicated because of the advanced age of the patient and existing cognitive impairment. Only published protocols should be used unless the patient is to be offered a trial that has either national or international support. Baseline neuropsychological tests should be carried out before treatment and repeated during and after treatment. The risks of cognitive impairment associated with the disease, with methotrexate - containing chemotherapy and with whole brain radiotherapy should be explained to patients and relatives when obtaining informed consent. Long-term survival, with current treatment regimes, is possible with PCNSL but this appears limited to patients less than 60 years of age at presentation (mostly patients less than 50 years of age).     

Advances in the Therapy of Primary Central Nervous System Lymphoma

Primary central nervous system lymphoma (PCNSL) is a rare type of non-Hodgkin's lymphoma (NHL) confined to the nervous system. The management of PCNSL is quite different from the usual treatment of either primary brain tumors or systemic NHL. First-generation chemotherapy regimens used successfully in systemic NHL are ineffective in PCNSL, in large part due to the existence of the blood-brain barrier. Whole-brain radiation therapy (WBRT) results in high response rates but rapid relapse, and this treatment is associated with delayed neurotoxicity in patients with PCNSL. The addition of methotrexate-based chemotherapy has improved survival and lessened toxicity for this patient population. Fundamental issues that remain unresolved in PCNSL include identification of the optimal chemotherapy regimen for newly diagnosed and relapsed PCNSL, the role of WBRT and intrathecal chemotherapy in the treatment of PCNSL, and the optimal management of intraocular lymphoma. Finally, the optimal clinical study design for this rare disease has yet to be defined and implemented.     

32例原发性中枢神经系统恶性淋巴瘤临床分析及文献复习

背景与目的:原发性中枢神经系统淋巴瘤(primarycentralnervoussystemlymphoma,PCNSL)发病率上升且预后很差。本研究目的是探讨免疫正常的中国人PCNSL的临床特征,评价大剂量甲氨蝶呤(HD-MTX)治疗PCNSL的疗效。方法:回顾性分析经病理证实的32例(中位年龄50岁)PCNSL患者的临床资料和治疗效果。2001年11月以前采用以CHOP方案为主、单用或联合全脑放疗的治疗方法,2001年12月以后采用以HD-MTX为主、单用或联合全脑放疗的治疗方法。结果:32例PCNSL患者中25例(78.1%)45岁以上;24例(75%)主要表现为颅内高压;25例(78.1%)单发病灶;32例患者均未见脑脊液细胞学阳性表现;28例(87.5%)为B细胞淋巴瘤,其中19例为弥漫性大B细胞淋巴瘤。32例中位随访期13.5个月(1～84个月),Kaplan-Meier分析总中位生存期26个月,2年生存率45.7%;HD-MTX联合放疗组患者完全缓解率61.1%,中位生存期在26个月以上,2年生存率65.1%,疗效明显优于非HD-MTX联合放疗组;log-rank检验显示乳酸脱氢酶正常、状态评分在0～1的患者生存期较长。结论:PCNSL多发于中老年人,颅内高压为主要表现,B细胞亚型占绝对优势。HD-MTX联合全脑放射治疗PCNSL有效和可行。     

Salvage treatment with temozolomide in refractory or relapsed primary central nervous system lymphoma and assessment of the MGMT status

High-dose methotrexate (HD-MTX) is effective in the initial treatment of primary central nervous system lymphoma (PCNSL). Because treatment options in patients with progressive or recurrent PCNSL are limited, prognosis is poor. Temozolomide, a well-tolerated oral alkylating agent that permeates the blood brain barrier (BBB), is effective against malignant glioma and recurrent PCNSL. The gene for the deoxyribonucleic acid (DNA) repair enzyme O⁶-methylguanine-DNA methyltransferase (MGMT), which is closely related to cellular sensitivity to alkylating agents, is inactivated by promoter hypermethylation. We evaluated the results of temozolomide treatment and the methylation status of the promoter region of the MGMT gene in 17 patients (median age 68 years) with refractory or relapsed PCNSL. They were immunocompetent and had received initial treatment with HD-MTX (3.5 g/m²) with or without irradiation. All were treated with temozolomide 150–200 mg/m², for 5 days in the course of 28 days; treatment was continued until disease progression. We observed five complete remissions, five partial responses (PRs) with stable disease (SD), and seven with disease progression. Median overall survival after the temozolomide treatment was 6.7 months. One patient manifested grade 3 neutropenia and thrombocytopenia. Eleven tumor specimens were available for MGMT analysis. MGMT promoter methylation (mMGMT) in the tumor tissue was found in 4 (36.4%), the other seven harbored a non-methylated MGMT promoter (nmMGMT). There was no statistically significant difference in median overall survival between patients with mMGMT (11.1 months) and nmMGMT (6.7 months) (P = 0.63). Although some patients were elderly and had been heavily pre-treated, temozolomide resulted in a complete response (CR) in 29% and was well tolerated without any major toxicity.     

Successful Management of Natalizumab-Associated Primary Central Nervous System Lymphoma through Autologous Stem Cell Transplant

Natalizumab is used as a second-line treatment for multiple sclerosis (MS). Some reports have linked natalizumab to primary central nervous system lymphoma (PCNSL), although few have described its management. A 45-year-old woman with Balo’s Concentric Sclerosis presented dizziness, vertigo accompanied by dysarthria, weakness on the left side and blurred vision to the right eye after the fourth dose of natalizumab. Magnetic resonance imaging (MRI) and a brain biopsy confirmed the diagnosis of PCNSL. The patient received modified PCNSL chemotherapy (MATRix protocol) followed by high-dose chemotherapy (HDC) supported by an autologous hematopoietic stem cell transplant (ASCT) as a consolidation therapy. Thirty months later, she is still in complete remission of her PCNSL and MS. In this case, whole brain radiotherapy was excluded because it may be associated with an increased risk of neurotoxicity in MS. ASCT was preferred because it has been shown to prevent disability progression in less advanced MS stages. Our patient is the second to receive an ASCT in this context and this option of treatment should be the preferred if the patient is eligible.     

Chemotherapy in newly diagnosed primary central nervous system lymphoma

Primary central nervous system lymphoma (PCNSL) accounts for only 3% of brain tumors. It can involve the brain parenchyma, leptomeninges, eyes and the spinal cord. Unlike systemic lymphoma, durable remissions remain uncommon. Although phase III trials in this rare disease are difficult to perform, many phase II trials have attempted to define standards of care. Treatment modalities for patients with newly diagnosed PCNSL include radiation and/or chemotherapy. While the role of radiation therapy for initial management of PCNSL is controversial, clinical trials will attempt to improve the therapeutic index of this modality. Routes of chemotherapy administration include intravenous, intraocular, intraventricular or intra-arterial. Multiple trials have outlined different methotrexate-based chemotherapy regimens and have used local techniques to improve drug delivery. A major challenge in the management of patients with PCNSL remains the delivery of aggressive treatment with preservation of neurocognitive function. Because PCNSL is rare, it is important to perform multicenter clinical trials and to incorporate detailed measurements of long-term toxicities. In this review we focus on different chemotherapeutic approaches for immunocompetent patients with newly diagnosed PCNSL and discuss the role of local drug delivery in addition to systemic therapy. We also address the neurocognitive toxicity of treatment.     

原发性中枢神经系统淋巴瘤的临床特征分析

 目的　分析原发性中枢神经系统淋巴瘤（PCNSL）的临床特征,探讨影响疾病的预后因素,并对不同的治疗方案进行评价。方法　回顾性分析初发PCNSL患者的临床资料、治疗经过及随访结果,应用Log-rank进行单因素分析,应用COX回归模型进行生存资料的多因素分析。结果　共收集PCNSL初发病例64例,中位年龄54.9岁,男性多于女性,肿瘤单发62 %（40／64）,深部病变占54 %（33／61）。在我科诊治的具有完整治疗资料的患者26例,其中19例患者初始治疗为单纯化疗,6例为全颅放疗（WBRT）后1个月进行化疗,1例患者初治时仅行WBRT。中位生存时间为17个月,血红蛋白≥9 g／L患者的生存时间长于血红蛋白＜9 g／L患者。年龄>60岁、性别、体能状态、病变部位等因素对预后无明显影响。应用含大剂量甲氨蝶呤（HD-MTX）或替尼泊苷的方案化疗者的预后优于未使用者,化疗联合放疗可能有助于改善患者的预后（χ2＝3.24,P＝0.07）,应用CHOP方案（环磷酰胺、多柔比星、长春新碱、泼尼松）、利妥昔单抗、鞘内注射化疗药物等与预后关系不大;多因素分析提示HD-MTX是影响PCNSL患者生存时间的独立有利因素,颅内病灶部位、病灶的多少、是否联合放化疗等均不是影响预后的独立因素。结论　PCNSL预后较差,应用HD-MTX、替尼泊苷等药物可改善患者的预后,贫血尤其中重度贫血患者预后不良。     

34例原发性中枢神经系统恶性淋巴瘤临床分析

目的：分析免疫功能正常的中国人原发性中枢神经系统淋巴瘤（PCNSL）的临床资料,探讨PCNSL的临床特征,评价大剂量甲氨蝶呤（HD-MTX）加全脑放疗（WBRT）治疗PCNSL的疗效.方法：回顾性分析34例经病理证实的PCNSL患者的临床资料以及治疗效果,Kaplan-Meier法分析患者生存期.结果：34例PCNSL患者中B细胞淋巴瘤31例（91.2%）,T细胞淋巴瘤3例（8.8%）;所有患者治疗后评价完全缓解率（CR）41.2%,2年生存率60.2%;病理类型和是否接受HD-MTX加放疗是影响PCNSL生存期的主要原因（P＜0.05）.结论：PCNSL以颅内高压为主要表现,B细胞亚型占绝对优势,具有独特的预后因素,HD-MTX联合放疗是PCNSL有效的治疗方法.