Primary central nervous system lymphoma: a report of 2 cases with clinicopathologic analysis and literature review

目的 探讨原发性中枢神经系统淋巴瘤(primary central nervous system lymphoma,PCNSL)的临床特点、病理诊断、治疗及预后.方法 对2例PCNSL进行免疫组化染色并复习相关国内外文献.结果 2例PCNSL,1例为间变性大细胞型-T细胞来源,另1例为弥漫性大B细胞型,HIV均阴性.PCNSL临床表现无特异性,颅内压增高、精神失常为常见症状,影像检查缺乏特征性改变,免疫表型以B细胞为主(87.5%～98%),大剂量氨甲蝶呤联合全脑放疗缓解率高.结论 PCNSL术前难以诊断,需依赖病理检查确诊,治疗困难,预后差.     

弥漫大B细胞淋巴瘤侵犯骨髓7例临床病理分析

目的探讨弥漫大B细胞淋巴瘤(diffuse large B-cell lymphoma, DLBCL)侵犯骨髓的临床病理特征、诊断、鉴别诊断及预后。方法收集7例DLBCL侵犯骨髓患者的临床资料、实验室检查、骨髓活检HE切片、免疫组化、治疗和预后,总结其临床病理特征。结果 DLBCL侵犯骨髓临床表现以发热和脾大最常见,骨髓涂片诊断阳性率为71.43%(5/7),流式细胞学诊断阳性率为42.86%(3/7),骨髓活检诊断阳性率为100%(7/7),染色体核型异常42.86%(3/7)。骨髓活检中异常细胞分布方式为弥漫型、间质型、混合型、结节型。免疫组化提示生发中心来源2例,1例表达CD5。纤维化2级6例(6/7,85.71%)。7例患者均呈侵袭性进展,2例未经治疗死亡。结论骨髓病理学及免疫组化检查对DLBCL侵犯骨髓诊断具有重要价值。     

原发性中枢神经系统淋巴瘤167例临床特征及病理类型构成分析

目的探讨原发性中枢神经系统淋巴瘤(primary central nervous system lymphomas,PCNSL)的临床特征、病理类型及构成比率,分析PCNSL的EBV感染率、c-MYC、BCL-2及BCL-6基因异常及免疫球蛋白基因重排情况。方法回顾性分析167例PCNSL的临床病理资料,总结其临床特征、病理类型及构成比率;原位杂交技术检测PCNSL中EBV编码的小RNA(EBER);荧光原位杂交(fluorescence in situ hybridization,FISH)技术检测c-MYC、BCL-2、BCL-6基因扩增及断裂重排情况;免疫球蛋白基因重排检测Ig H和Ig K。结果 PCNSL占所有淋巴瘤的0. 95%;大多为单一病灶,约占60%,且多位于浅部脑组织(66. 93%)及小脑幕上(87. 40%),最常累及额叶和颞叶,51～60岁为发病高峰期。PCNSL中霍奇金淋巴瘤(Hodgkin’s lymphoma,HL)仅1例;非霍奇金淋巴瘤(non-Hodgkin’s lymphoma,NHL) 166例:B细胞淋巴瘤占158例,其中弥漫大B细胞淋巴瘤(diffuse large B-cell lymphoma,DLBCL)最常见; T/NK细胞淋巴瘤8例,其中以ALK阳性间变大细胞淋巴瘤多见。13例原发性中枢神经系统弥漫大B细胞淋巴瘤(primary central nervous system-diffuse large B-cell lymphoma,PCNS-DLBCL) EBER均阴性; 11例PCNS-DLBCL中1例c-MYC基因拷贝数增加,未见断裂重排; 1例BCL-2基因拷贝数增加,未见断裂重排; 2例BCL-6基因拷贝数增加,1例断裂重排;免疫球蛋白Ig H和Ig K呈克隆性重排。结论 PCNSL病理类型多样,HL和NHL均可发生,以PCNSDLBCL最多见,T细胞来源的淋巴瘤以ALK阳性间变大细胞淋巴瘤多见,51～60岁为发病高峰期。     

原发性中枢神经系统淋巴瘤临床病理及病因学分析

目的 探讨原发性中枢神经系统淋巴瘤(primary central nervous system lymphoma, PCNSL)的临床病理特征、预后指标及病因学.方法 复习39例PCNSL患者的临床资料,同时进行免疫组化、原位杂交检测EBER及PCR检测bcl-2/JH基因重排,并对临床资料、免疫标志物与预后的关系进行分析.结果 34例PCNSL患者的3年生存率为46.4%,5年生存率为27.1%,年龄≥60岁及病变部位深对预后不利(P=0.009和P=0.027),bcl-6阳性表达者的生存率高于阴性表达者(P=0.044),但多因素Cox回归分析显示,进入回归方程的为年龄因素.CD10/bcl-6/MUM-1/CD138分型和治疗方法对预后的判断无显著性差异(P＞0.05).39例患者EBER原位杂交均为阴性,bcl-2/JH基因重排5例阳性(12.8%),其中3例为CD10阳性病例.结论 PCNSL是一种少见的高侵袭性结外非霍奇金淋巴瘤,年龄因素是判断预后的独立性指标,CD10/bcl-6/MUM-1/CD138分型未发现有预后意义,但显示PCNSL的同质性较高,可能是弥漫性大B细胞淋巴瘤的一种亚型.EB病毒感染与PCNSL的病因无相关性.     

原发骨髓淋巴瘤4例临床病理学分析

目的探讨原发骨髓淋巴瘤(PBML)临床与病理特征, 以提高认识, 避免误诊和漏诊。方法回顾性分析4例PBML的临床表现、病理学特点、免疫表型及治疗与预后特征, 结合文献报道进行总结。结果 4例PBML患者年龄67～81岁(平均72.5岁), 均为女性。1例出现B症状, 2例表现为乏力、纳差, 1例表现为活动后憋喘。骨髓内肿瘤细胞占比30%～80%;瘤细胞体积中等偏大, 2例以中心母细胞为主, 1例以浆样形态大细胞为主, 1例以免疫母细胞为主;2例瘤细胞弥漫型浸润, 2例为弥漫型和间质型浸润模式。4例均表达常用B细胞标志物(CD20、PAX5等), CD10均阴性, 2例表达bcl-6, 1例表达MUM1, 3例表达bcl-2, 2例表达MYC。2/3例行流式细胞检测发现异常成熟B细胞群, 其中例1为双克隆性, 结合形态和免疫组织化学诊断为B细胞淋巴瘤难以分类。另3例诊断为弥漫性大B细胞淋巴瘤, 包括1例生发中心B细胞型(GCB型), 2例非GCB型伴MYC/bcl-2双表达。结论 PBML多无特异性临床症状, 早期诊断困难, 充分认识临床病理及免疫表型特点, 并严格排除系统性淋巴瘤累及...     

原发性中枢神经系统淋巴瘤VEGF、MVD与影像学对比研究

目的 :分析原发性中枢神经系统淋巴瘤 (PCNSL)的VEGF表达分布和测量其微血管密度 (MVD) ,旨在提高PCNSL早期诊断率和判断预后 ,同时为影像学提供理论依据。方法 :对 2 2例PCNSL临床 (包括影像学 )病理资料分析 ,同时行VEGF及CD34免疫组化标记 ,并测量MVD ,以 12例胶质瘤作为对照。结果 :2 2例PCNSL中单发者 17例 (占 77 2 7% ) ;多发者 4例 ,共有病灶 10处 ;1例为弥漫浸润型。肿瘤位于脑白质深部者 15例 (占 5 5 5 6 % )、脑表面及灰白质交界区者 8例、胼胝体者4例。CT示肿瘤为边界清楚的高密度结节或肿块。组织病理学示瘤细胞弥漫分布 ,瘤细胞大小较一致 ,胞质少 ,核大 ,可见瘤细胞围绕血管呈“袖套样”浸润。淋巴瘤MVD值 (2 1 8± 11 6 )与恶性胶质瘤组 (44 4± 16 8)的差异有非常显著性 (t =3 374 ,P <0 0 1)。VEGF表达无特异性 ,与对照组比较无统计学意义。结论 :病理学基础决定了影像学的特征。血管生成活性的不同 ,有助于PCNSL与恶性胶质瘤的鉴别 ,并对其预后的判断有一定帮助 ,VEGF可能是恶性肿瘤重要的促血管生成因子 ,但对于鉴别诊断无特异性。     

胸腺癌与B3型胸腺瘤的临床病理研究

目的探讨胸腺肿瘤的临床病理特征。方法复习9例胸腺癌和21例B3型胸腺瘤，全部临床病史、影像学、实验室资料和肿瘤分期。标本常规病理制片、光镜观察和免疫组化标记。结果男17例，女13例，年龄28～70岁(中位43岁)，病程平均7．4个月。19例伴重症肌无力；9／22例纵隔X线检查未见异常；20／22例CT诊断胸腺瘤；肿瘤临床分期Ⅱ期1例，Ⅲ期22例，Ⅳ期7例，全部肿瘤呈浸润性生长，包括肺(22例)、心包(15例)、无名静脉(10例)和主动脉(5例)等，27例肿瘤完全切除。组织学表现低分化鳞状细胞癌(4例)，梭形细胞型鳞癌(2例)，大细胞未分化癌(2例)，基底细胞样癌(1例)和B3型胸腺瘤(21例)。肿瘤细胞以实性、索状浸润生长为其主要特征，缺乏核分裂象，部分肿瘤伴B1和B2型胸腺瘤成分；标记显示梭形细胞型鳞状细胞癌缺乏神经内分泌分化。结论胸腺癌和B3型胸腺瘤好发于40岁后中年人，肿瘤完全浸润性生长，术前多数患者系肿瘤的临床Ⅲ期，CT检查是诊断该病的主要手段，90％患者可行外科治疗，确诊依赖于病理组织学检查。     

MTX相关性原发中枢神经系统淋巴瘤1例并文献复习

目的探讨MTX相关淋巴增殖性疾病(methotrexateassociated lymphoproliferative disorders,MALD)的临床病理特征、鉴别诊断及预后。方法回顾性分析1例MTX相关原发中枢神经系统淋巴瘤(primary central nervous lymphomas,PCNSL)的临床病理资料并复习相关文献。结果患者男性,61岁。因行走不稳伴右下肢乏力半个月入院,银屑病7~8年,每天口服MTX 0.25 mg。头颅MR示左侧扣带回及胼胝体体部占位性病变。脑肿物病理结果:非霍奇金淋巴瘤,B细胞性;弥漫大B细胞淋巴瘤(非生发中心型)免疫亚型;老年性EBV~+弥漫大B细胞淋巴瘤。EBER检测阳性。免疫表型:CD20、CD79a、CD30、BCL-2、CD43均弥漫强阳性,MUM1(70%阳性),Ki-67(90%阳性),BCL-6(10%阳性)。CD3、CD5、CD10、CD15、EMA、CD4、CD2、ALK、CD138、TDT、Cyclin D1、CD56、TIA-1、c-myc均阴性。BCR毛细管电泳法显示:Ig H基因可见克隆性重排;Ig K、Ig L基因未见克隆性重排。BCL-2/Ig H、BCL-6 BA、c-myc BA荧光原位杂交结果均阴性。结论 MALD临床少见,极易误诊。熟悉其临床病理表现及免疫表型特征,结合临床病史,有助于正确诊断。     

原发性睾丸弥漫性大B细胞淋巴瘤的临床病理及预后

目的观察原发性睾丸弥漫性大B细胞淋巴瘤的临床病理、免疫表型特征及患者存活情况,探讨该肿瘤的病理诊断、鉴别诊断及预后。方法按WHO（2001）淋巴瘤分类标准收集14例原发性睾丸弥漫性大B细胞淋巴瘤,中位年龄62岁,按AnnArbor分期标准,Ⅰ期10例,Ⅱ期3例,Ⅳ期1例。11例有随访资料,其中3例存活,最长存活时间86个月;8例死亡,存活时间5～19个月,中位存活时间为11个月。总结14例的组织病理学、免疫表型特征,并进行存活分析。结果单侧睾丸无痛性肿大是最常见的临床表现。形态学变型全部为中心母细胞性。免疫分型,生发中心样B细胞型（GCB型）1例,非生发中心样B细胞型（non—GCB型）13例。10例p53蛋白表达阳性,肿瘤细胞增殖活性高,6例肿瘤细胞表达bcl-2蛋白。存活分析表明,1、2、5年生存率分别为45．5％、17．0％、17．0％。结论原发性睾丸弥漫性大B细胞淋巴瘤多为外周活化的B细胞起源,预后差,易复发和转移;病理活检加免疫表型检测对肿瘤的诊断和鉴别诊断有重要作用。     

原发性中枢神经系统淋巴瘤的临床病理、免疫表型及其与EB病毒相关性研究

目的：观察原发性中枢神经系统淋巴瘤（PCNSL）的临床病理、免疫表型及其与EB病毒的关系。方法：搜集25例PCNSL的临床资料并随访,应用单克隆抗体UCHL－1、L26、k、λ胶质纤维酸性蛋白（GFAP）和CS1－4行免疫组织化学染色,EB病毒寡核苷酸探针（EBER1／2）原位杂交,研究其免疫表型和EB病毒感染情况。结果：25例PCNSL均为B细胞淋巴瘤,EBER1／2原位杂交25例中仅2例（8％）出现阳性。结论：本组PCNSL均为B细胞激性。且与EB病毒呈低相关性。     

肠道原发性非霍奇金淋巴瘤32例的临床与病理学分析

目的研究肠道原发性非霍奇金淋巴瘤(NHL)的临床表现、病理特点及预后因素。方法复习32例肠道原发性NHL的临床资料、大体标本、HE切片及免疫组织化学染色结果,按2001年WHO淋巴造血系统肿瘤的标准重新进行分类。结果B细胞性NHL 21例,其中弥漫大B型15例,套细胞型2例,滤泡型1例,Burkitt淋巴瘤1例,黏膜相关淋巴组织淋巴瘤2例。T细胞NHL 10例(其中肠病相关型NHL2例和非肠病相关型NHL8例),组织细胞NHL1例。诊断时,9例为Ⅰ～Ⅱ期,23例为Ⅲ～Ⅳ期。随访4—168个月,死亡15例。B细胞NHL死亡率为7／21例(33％),T细胞NHL的死亡率为8／10。Ⅱ期死亡2例,均为T细胞NHL。Ⅲ-Ⅳ期死亡13例,占死亡数的86．6％。Cox多因素分析显示,最重要的预后因素是肿瘤的分期及肿瘤类型(分期P=0．002、肿瘤类型P=0．032)。结论肠道原发性NHL以弥漫大B型最多见。以结肠最多见,其次为小肠和回盲部,直肠最少。就诊时65．6％为Ⅲ-Ⅳ期患者。比较同期T、B细胞NHL,T细胞NHL预后差,死亡率高。分期越高预后越差,黏膜相关淋巴组织淋巴瘤等恶性度较低的淋巴瘤预后较好。     

Primary central nervous system lymphoma

Primary central nervous system lymphoma (PCNSL) is an uncommon extranodal non-Hodgkin lymphoma. Its incidence has increased during the last 3 decades and has been reported in both immunocompromised and immunocompetent patients. Immunocompromised patients are affected at a younger age compared with immunocompetent patients. It presents with raised intracranial pressure and focal neurologic and neuropsychiatric symptoms. The lesions are typically solitary. The majority of the lesions are located in the periventricular area, whereas in a few cases they are located in the supratentorial area. Diffuse large B-cell lymphomas constitute most PCNSLs, whereas T-cell, low-grade, anaplastic, and Hodgkin lymphomas are rarely encountered. The morphology of PCNSL shows a characteristic angiocentric pattern and is positive for B-cell markers by immunohistochemistry. The differential diagnosis of PCNSL includes central nervous system gliomas, metastatic tumors, demyelinating disorders, subacute infarcts, and space-occupying lesions due to an infectious etiology. The understanding of the molecular mechanisms involved in the pathogenesis of PCNSL and the identification of molecular biomarkers have lagged behind that of systemic nodal lymphomas. Primary central nervous system lymphomas are treated with combined radiotherapies and chemotherapies. The prognosis for PCNSL is worse than for other extranodal lymphomas.     

Primary CNS lymphomas. Morphology and diagnosis

Diagnostically primary central nervous system lymphomas (PCNSL) have to be differentiated from glioblastoma and brain metastases. Histologically the overwhelming majority of PCNSL is represented by diffuse large B-cell lymphomas, in this series with a BCL6 expression in 80% of the cases detected by immunohistochemistry. Stereotactic biopsy is the method of choice in establishing the definite diagnosis and intraoperative smear cytology will detect the lymphoid blasts. To confirm the B-cell lineage, immunohistochemistry is needed (CD20, CD79a). Small reactive T-lymphocytes and monohistiocytic cells and activated "microglia" are found within and at the periphery of PCNSL foci. The infiltrated brain tissue shows partially pleomorphic reactive astrocytes that can be confused with neoplastic astrocytes, especially in small specimens. In contrast to high-grade gliomas, however, PCNSLs do not show endothelial proliferations. Subtypes or variants of diffuse large B-cell lymphomas can also be observed in cases of PCNSL: the anaplastic variant with large multinucleated tumour cells resembling Reed-Sternberg cells, T-cell rich B-cell lymphoma and intravascular B-cell lymphoma with primary manifestation within the brain or the spinal cord. HIV/AIDS-associated PCNSLs are characterised by large plasmoblastic or small Burkitt-like cells and tumour necrosis. Primary leptomeningeal large B-cell lymphomas do occur very rarely and are diagnosed by cerebrospinal fluid cytology.     

Clinical pathology of primary central nervous system lymphoma in HIV-positive patients-a 41 Chinese patients retrospective study

ObjectivesThe purpose of this study was to investigate the clinicopathological characteristics of primary central nervous system lymphoma (PCNSL).MethodsWe collected 41 PCNSL formalin-fixed, paraffin-embedded (FFPE) samples from human immunodeficiency virus (HIV)-positive patients and performed HE (haematoxylin-eosin) staining, immunohistochemistry (IHC) staining, in situ hybridization, fluorescence in situ hybridization (FISH). Real-time quantitative polymerase chain reaction (RT-qPCR) was performed in 9 cases of FFPE samples. Meanwhile, we analysed the clinical pathological significance of the results.ResultsSeven patients had diffuse large B-cell lymphoma (DLBCL) with germinal centre B-cell (GCB)-like DLBCL, 32 had activated B-cell (ABC)-like DLBCL, and 2 had Burkitt lymphoma (BL). GCB-like DLBCL patients were older at onset (P = 0.040).A lower CD4⁺ T-cell count and a decrease in cerebrospinal fluid (CSF) glucose content were more frequent in ABC-like DLBCL (P = 0.012, P = 0.006). Overexpression of P53 was more in ABC-like DLBCL (P = 0.041). 73.2 % cases were Epstein–Barr encoding region (EBER) positive, which was more likely in ABC-like DLBCL patients (P = 0.037). EBV DNA were detected in 5/7 EBER-negative DLBCL cases and none (0/2) of the BL cases. All the cases were negative for HHV8 staining. None of the 7 Double expressor lymphoma (DEL) cases had BCL2, BCL6, or c-MYC genetic rearrangements.ConclusionsHIV-related PCNSL showed unique clinical pathological significance. None of EBV detected in HIV-related BL and without HHV8 infectious are new sights in our single-center study of Chinese HIV-related PCNSL patients.     

Primary central nervous system lymphoma secretes monocyte chemoattractant protein 1

The majority of primary central nervous system lymphomas (PCNSL) are diffuse large B-cell lymphomas. Histologically, reactive T lymphocytes and monohistiocytic cells are found within PCNSL tissue. To clarify the mechanisms of the cellular infiltration, the presence of monocyte chemoattractant protein (MCP-1) was investigated in biopsy samples of 19 cases of PCNSL by means of immunohistochemical staining, double staining with a confocal laser microscope, and Western blot analysis. MCP-1 expression was observed in all PCNSL immunohistochemically. Western blot analysis showed that the concentration of MCP-1 in PCNSL was as high as that in a metastatic brain tumor. In normal brain tissue, MCP-1 was not detected. Confocal laser microscope revealed MCP-1 signals were present in the cells with CD20, a B-cell marker. We concluded that lymphoma cells produced MCP-1, which is an additional cytokine involved in the pathogenesis of PCNSL.     

Cytomorphology of primary CNS lymphoma: Review of 23 cases and evidence for the role of EBV

Primary non-Hodgkin's lymphoma of the central nervous system (PCNSL) has recently increased in incidence, due primarily to an enlarging immunosuppressed patient population. The pathogenetic role of Epstein-Barr virus (EBV) is of interest due to its established role in other lymphoproliferative disorders in immunosuppressed patients. Twenty-three cases of histologically confirmed PCNSL with corresponding cytology were identified, all obtained under stereotactic guidance. Twenty patients were human immunodeficiency virus (HIV) positive, two were HIV negative, and one was of unknown status. Papanicolaou-stained slides were selected from each case and evaluated for the presence of EBV RNA via in situ hybridization (ISH) utilizing a biotinylated probe specific for EBER 1 RNA, and detected by a conventional streptavidin-peroxidase system. The cases included immunoblastic (12), large cell (10), and mixed small and large cell lymphoma (1). The predominant immunophenotype was B-cell (19), although T-cell (2) and biphenotypic (1) cases were also identified. ISH showed nuclear positivity for EBV RNA in 19 of 23 cases (83%). This study confirms the presence of EBV in PCNSL in immunosuppressed patients and implies a potential etiologic role. The ability to demonstrate EBV RNA in cytologic preparations by ISH also raises the possibility of early identification of high-risk patients through detection of EBV-infected lymphocytes in CSF specimens. Diagn Cytopathol 1996; 14:114–120. © 1996 Wiley-Liss, Inc.     

Selective central nervous system tropism of primary central nervous system lymphoma

Primary Central nervous system lymphoma (PCNSL) is most frequently a diffuse large B cell lymphoma (DLBCL), which is confined to the Central nervous system (CNS). We performed an experiment in which lymphoma cells from a PCNSL patient were implanted subcutaneously in an athymic mouse. The lymphoma cells were shown to home to the CNS with histologic evaluations of the brain showing multiple large B cells in blood vessels consistent with intravascular large B cell lymphoma (IVL). We did not find any evidence of lymphoma at the site of implantation or other locations. The findings are consistent with highly selective tropism of PCNSLforthe CNS and its vasculature.     

Histological and immunohistochemical studies on primary gastrointestinal lymphomas.

To study the characteristics and histogenesis of the malignant lymphomas derived from the gastrointestinal mucosa, histologic and immunohistochemical analyses were performed on a series of 28 malignant lymphomas of the gastrointestinal tract. By cytomorphologic classification, there were two small lymphocytic lymphomas, one small cleaved cell lymphoma, two mixed small cleaved and large cell lymphomas, 17 large cell lymphomas, one small noncleaved cell lymphoma, three immunoblastic lymphomas, and two lymphoblastic lymphomas. This distribution of histologic types was compatible with that of nodal lymphoma. The lymphomas with poor prognostic histology (23 cases) outnumbered those with favorable prognosis (five cases). Three of 28 cases (one in the stomach and two in the small intestine) had cytologic features consistent with centrocytoid cell lymphoma of the mucosa associated lymphoid tissue and were large cell lymphomas. Immunophenotypically, 23 cases expressed B-cell markers (82.1%) and three cases reacted with T-cell markers. Two cases did not react with either T-cell or B-cell markers. True histiocytic lymphomas were not identified. Gastric lymphomas (nine cases) and colorectal lymphomas (three cases) were of B-lymphocyte origin whereas T-cell lymphomas were noted in the small intestine (two cases) and ileocecal region (one case). Three cases of centrocytoid lymphoma were of B-lymphocyte origin. Histologically B-cell lineage lymphomas were evenly distributed on various histologic subtypes but all T-lineage lymphomas belonged to the large cell type. The two cases with undetermined phenotype were lymphoblastic lymphomas histologically. This study showed that the primary GIT lymphomas, mostly of B-cell lineage, were not cytomorphologically distinctive from the nodal lymphomas.(ABSTRACT TRUNCATED AT 250 WORDS)     

原发性中枢神经系统非霍奇金淋巴瘤临床分析

目的：探讨原发性中枢神经系统非霍奇金淋巴瘤(primary central nervous system non-Hodgkin’s lymphoma,PCNSL)的临床特征、治疗及预后。方法：回顾性分析4例原发性中枢神经系统淋巴瘤的临床资料和治疗疗效观察,并随访患者的复发率及生存情况。结果：4例原发性中枢神经系统淋巴瘤患者均为男性,发病年龄46~76岁,中位58.75岁。病灶分别位于脑干双侧丘脑低节、右侧脑室、右侧枕叶、右侧小脑,以头痛引起的颅内高压为主要临床症状。4例患者均术后给予大剂量MTX(HD-MTX)为主的化疗+全脑放疗;随访3年,1例复发术后死亡,3例存活。结论：原发性中枢神经系统淋巴瘤多发于中老年男性,颅内高压为主要临床症状,B细胞亚型占绝对优势。HD-MTX联合全脑放射治疗原发性中枢神经系统淋巴瘤有效可行,可提高患者的远期生存率。     

原发性全身皮肤弥漫大B细胞淋巴瘤1例

目的:进一步认识原发性全身皮肤弥漫大B细胞淋巴瘤(primary cutaneous diffuse large B-cell lymphoma,PCLBCL)的临床表现、病理特征及其预后评估.方法:收集1例PCLBCL病例资料及相关参考文献,观察其临床表现、临床病理学特征、免疫组织化学染色及治疗效果判断,并进一步评价预后.结果:PCLBCL常见发病部位为双下肢,其次为躯干和头部.本例病例病理结果考虑皮肤非霍奇金弥漫大B细胞淋巴瘤,生发中心外活化B细胞起源.因患者高龄,一般情况差,治疗后效果欠佳.结论:PCLBCL预后取决于患者的年龄、皮肤损伤部位、数量和疾病持续时间及病理特征.