原发气管弥漫大B细胞淋巴瘤一例并文献复习

目的:探讨原发气管弥漫大B细胞淋巴瘤（DLBCL）的临床特点、实验室检查、治疗及预后。方法:回顾性分析郑州大学第一附属医院收治的l例原发气管DLBCL合并干燥综合征（SS）患者的临床资料,并结合文献进行复习。结果:该患者明确诊断为DLBCL（非生发中心B细胞型）,Ⅰ期,低危组,国际预后指数（IPI）评分0分;SS。给予R-CHOP方案化疗2个周期后达到部分缓解,4个周期后达到完全缓解,共予6个周期化疗及1次局部放疗。随访24个月,患者一般情况尚可,临床无不适主诉,PET-CT未发现代谢异常细胞。结论:原发气管DLBCL非常少见,发病原因可能与SS等自身免疫性疾病有关,临床表现无特异性,易误诊,诊断主要靠病理学检查,治疗主要包括气管镜下治疗等手术治疗、联合放化疗等,预后可能与临床分期、IPI评分、肿瘤细胞来源及治疗等有关。     

原发心脏弥漫大B细胞淋巴瘤一例并文献复习

目的:探讨原发心脏弥漫大B细胞淋巴瘤（DLBCL）的临床特征、诊疗及预后。方法:回顾性分析安徽医科大学第一附属医院2018年11月收治的1例原发心脏DLBCL伴房室传导阻滞患者的诊疗经过,并复习相关文献。结果:患者,女性,48岁,初期临床表现为反复活动后胸闷、气喘,后突发晕厥,行心脏肿瘤切除术+双腔起搏器植入术,术后病理提示DLBCL,予以R-CHOP方案化疗8次。随访至截稿前,患者病情处于稳定状态。结论:原发心脏DLBCL恶性程度高,病情进展迅速,临床表现缺乏特异性,多通过尸检或术后病理发现;治疗上以化疗联合放疗为主,手术辅助治疗;总体预后不佳。     

原发心脏弥漫大B细胞淋巴瘤一例并文献复习

目的 了解原发心脏弥漫大B细胞淋巴瘤（DLBCL）的临床特点、治疗及预后.方法 结合1例患者临床资料同时复习国内外相关文献,对原发心脏DLBCL的临床特点、治疗及预后进行分析.结果 患者初表现为胸闷、双下肢水肿、心脏衰竭,就诊心外科,在全身麻醉下行右房肿物摘除术并行病理检查确诊为原发心脏DLBCL,采用CHOP方案化疗1个疗程后出现阵发性室上性心动过速,并经治疗得到纠正,已接受3个疗程化疗,处于化疗间歇期.结论 原发心脏DLBCL一般对化疗敏感,但非特异性的临床表现及肿瘤的特殊位置致其预后差.     

血管免疫母细胞性T细胞淋巴瘤并发EB病毒阳性弥漫大B细胞淋巴瘤二例并文献复习

目的:探讨血管免疫母细胞性T细胞淋巴瘤（AITL）并发EB病毒（EBV）阳性弥漫大B细胞淋巴瘤（DLBCL）患者的临床病理特征、治疗及预后。方法:回顾性分析解放军总医院第五医学中心2例AITL并发EBV阳性DLBCL患者的临床资料,并进行文献复习。结果:例1为混合淋巴瘤（CL）患者,以低热伴全身浅表淋巴结肿大起病,右侧腋窝肿物活组织检查示AITL并发EBV阳性DLBCL,予以8个周期化疗后达不确定的完全缓解;后续应用西达本胺维持治疗,仍生存中。例2为不一致性淋巴瘤（DL）患者,以皮下结节起病,后出现浅表淋巴结进行性肿大;皮下结节病理检查诊断为DLBCL,右腹股沟淋巴结病理检查诊断为AITL;接受7个周期化疗,因合并噬血细胞综合征而死亡。结论:AITL合并EBV阳性DLBCL罕见,临床症状主要以AITL的表现为主,存在T细胞及B细胞免疫表型特征,预后差,治疗方案主要依据预后较差的淋巴瘤进行选择。     

原发胃肠道淋巴瘤24例

 目的　分析胃肠道淋巴瘤的诊断及治疗方法。方法　对2004年1月至2008年12月诊治的24例原发胃肠道非霍奇金淋巴瘤（NHL）患者的临床资料进行回顾分析。结果　24例中胃淋巴瘤16例（66 %）,其中弥漫大B细胞淋巴瘤（DLBCL）9例,黏膜相关样淋巴组织（MALT）淋巴瘤7例;肠道淋巴瘤8例（33 %）,其中DLBCL 6例,MALT淋巴瘤2例。经胃镜确诊17例,手术确诊7例。DLBCL患者接受CHOP、ECHOP及CHOP样化疗方案,7例经手术确诊的患者有6例术后接受了联合化疗,1例患者自动出院。7例MALT淋巴瘤患者予以CHOP联合抗幽门螺杆菌（HP）治疗,2例予以FMD方案化疗。5例肠道淋巴瘤患者化疗结束后接受累及野放疗。24例患者中3例死亡,1例自动出院失访。目前20例患者生存,其中12例患者完全缓解（CR）,7例患者部分缓解（PR）,1例患者稳定（SD）,化疗有效率79 %,生存率83 %。结论　内窥镜活检目前是胃肠道淋巴瘤诊断最可靠的方法,也是漏诊率、误诊率最低的方法,化疗对胃肠道淋巴瘤的作用已取得共识,具有广泛的适应证,对于已有播散的晚期NHL以及生长迅速的高度侵袭性淋巴瘤患者应首选化疗,胃淋巴瘤手术联合化疗效果并不优于化疗联合放疗。     

10例艾滋病相关恶性淋巴瘤临床分析

总结北京地坛医院收治的艾滋病合并恶性淋巴瘤患者的临床特点及诊治经过。方法:回顾性分析10例艾滋病合并恶性淋巴瘤患者的特点,从临床表现、病理检查及免疫水平、EBV抗体检查结果、并发症及治疗情况、转归等多角度进行分析。结果:10例患者主要临床表现有间断性发热2例,颈部肿物伴发热3例,腋窝淋巴结肿大2例,腹痛腹胀伴发热3例;病理诊断弥漫大B细胞淋巴瘤（diffuse large B-cell lymphoma,DLBCL）7例,Burkitt淋巴瘤3例;CD4细胞计数<200/μL的8例,>200/μL者2例;EBV-IgM抗体阴性7例,未查3例;6例采取了不同方案和疗程的化疗,预后有差异,其中1例Burkitt淋巴瘤VP化疗后予以交替CODOXM与IVAV方案化疗3个周期,1例胃部伴肝转移DLBCL行R-CHOP化疗5次,后改为R-MINE、MINE化疗,1例肾上腺DLBCL患者CHOP化疗6个周期,3例DLBCL患者采取CHOP化疗1～2个周期,4例放弃治疗;化疗期间发生不同程度的感染、骨髓抑制、消化道出血、肾功能损害等不良反应。6例进行了高效抗逆转录病毒治疗（highly active antiretroviral treatment,HAART）,4例未行HAART;死亡6例,好转3例,1例自动出院。结论:艾滋病合并恶性淋巴瘤的患者临床表现多样,免疫力低下,多部位侵犯,就诊时已至淋巴瘤中晚期,化疗效果一般,并发症多,HAART不能明显改善患者预后,总体预后差。      

一种新的淋巴瘤亚型:bcl-2和myc易位的双击淋巴瘤

 双击淋巴瘤（DHL）特征介于弥漫大B细胞淋巴瘤（DLBCL）和伯基特淋巴瘤（BL）之间,通常伴有myc基因断裂和其他重现性染色体断裂的疾病,常见myc和bcl-2基因的易位。其临床表现具有乳酸脱氢酶升高、骨髓受累、Ann Abort分期晚期、B症状、结外受累、侵犯中枢神经系统等特征。因与DLBCL和BL有部分重叠,所以依靠病理诊断很难将其区分出来,目前主要的诊断方法为G显带染色体核型分析、荧光原位杂交（FISH）检测以及免疫组织化学技术。DHL对于DLBCL的标准化疗方案反应较差,预后不佳,中位生存期仅为0.2～1.5年。目前DHL尚无较好的治疗方法,主要方案为RCHOP、RICE、RCVD、甲氨蝶呤预防中枢神经系统受累、大剂量化疗联合骨髓移植等。     

弥漫性大B细胞淋巴瘤继发中枢神经系统侵犯的诊疗进展

弥漫性大B细胞淋巴瘤（diffuse large B-cell lymphoma，DLBCL）继发中枢神经系统（central nervous system，CNS）侵犯是一种严重的临床并发症，治疗方法有限，预后普遍较差。随着利妥昔单抗的广泛应用，其发生率呈降低趋势，但是脑实质受累越发多见，病理活检在诊断中则越发重要。在高危因素鉴定方面需要结合临床与分子生物学因素综合评估。以大剂量甲氨蝶呤为主的全身化疗作为主流的预防策略，其作用尚存争议。伊布替尼等新药、新疗法的治疗价值得到进一步探索。本文旨在对近年来DLBCL继发CNS侵犯的诊疗进展予以综述。      

原发中枢神经系统淋巴瘤诊疗进展

原发中枢神经系统淋巴瘤是一种少见的中枢神经系统恶性肿瘤,以大剂量甲氨蝶呤为基础方案诱导化疗是目前一线治疗,随后全脑放疗作为巩固治疗.迟发神经认知功能障碍出现,尤其是老年人,使缓解患者是否需要全脑放疗或减量放疗需要进一步明确.利妥昔单抗治疗PCNSL,初步研究表明美罗华可能给患者带来获益,但研究证据水平较低.HDC/ASCT对于治疗复发性或难治性PCNSL虽有疗效但毒副作用较大,临床价值尚存争议.     

大剂量甲氨蝶呤联合放疗治疗１９例原发性中枢神经系统淋巴瘤的临床观察

目的　观察大剂量甲氨喋呤联合放疗治疗原发性中枢神经系统淋巴瘤的疗效。方法　２００３年１月至２００８年２月我院经手术或立体定向活检病理证实的１９例原发性中枢神经系统淋巴瘤患者,予大剂量甲氨蝶呤化疗５～８个周期及阿糖胞苷＋地塞米松鞘内注射１２～１６次,序贯全脑放疗３０Ｇｙ,缩野１０Ｇｙ。结果　１９例患者中位生存期为４４个月,预计５年生存率为２８.６％。毒副作用以骨髓抑制（１００％）及胃肠道反应（7７％）多见,其次为肝肾功能损害、口腔炎及脱发等。毒副反应多数可耐受。结论　大剂量甲氨蝶呤化疗及阿糖胞苷＋地塞米松鞘内注射序贯放疗治疗原发性中枢神经系统淋巴瘤的疗效满意,安全性好。     

介于弥漫大B 细胞淋巴瘤和伯基特淋巴瘤之间的未分类淋巴瘤

目的:分析介于弥漫大B 细胞淋巴瘤和伯基特淋巴瘤之间的未分类的B 细胞淋巴瘤（B-cell lymphoma ,unclassifiable,with features intermediate between DLBCL and Burkitt lymphoma,DLBCL/BL）的临床特点、治疗与预后,增加对该病的认识。方法:收集郑州大学第一附属医院2013年1 月至2014年12月收治的13例DLBCL/BL患者临床病理资料,采用Kaplan-Meier 法进行生存分析,采用Logrank 检验对临床分期、年龄、LDH 水平、IPI 评分、初治化疗方案等进行单因素分析。结果:13例患者中12例存在结外侵犯,13例患者的中位OS为10个月,中位PFS 为6 个月。单因素分析显示IPI 评分、LDH 水平与预后有统计学相关性,行CHOP、CHOP 样与高强度化疗方案患者之间生存差异具有统计学意义（P = 0.054）。 结论:DLBCL/BL恶性程度高,生存期短,结外侵犯多见,对CHOP 及CHOP 样方案治疗反应差,高强度化疗可能改善预后,IPI 评分≥ 3 分及 LDH 升高是其不良预后因素。      

Primary mediastinal B-cell lymphoma

Primary mediastinal B-cell lymphoma (PMBCL) is a sub-type of the heterogeneous diffuse large B-cell lymphoma category, and comprises approximately 5% of all non-Hodgkin's lymphomas (NHL). It was first recognized as a distinct clinico-pathologic entity 20 years ago, and recent work has further characterized specific molecular features. Gene expression profiling has suggested a partial overlap with nodular sclerosing Hodgkin lymphoma (HL), with which it shares some clinical features. The optimal management remains a matter of debate. There is uncertainty as to whether weekly alternating chemotherapy regimens may be more effective than CHOP, whether consolidation radiotherapy (RT) to the mediastinum is always required, whether PET scanning can be used to determine this, and whether the use of rituximab as part of initial therapy will change the answers to these questions. The International Extranodal Lymphoma Study Group (IELSG) 26 clinicopathologic study of PMBCL, which has recently opened, represents a first attempt to gather data prospectively on some of these issues.     

Diffuse large B-cell lymphoma

Diffuse large B-cell lymphoma (DLBCL) is one of the most common subtypes of non-Hodgkin lymphoma. It is a heterogeneous disease, and a distinctive subgroup of patients with different treatment outcome can be identified based on clinical and molecular prognostic factors. Cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) chemotherapy has been the standard systemic therapy for this disease with a cure rate of 40% to 50%, although, more recently, the addition of rituximab has been shown in phase III trials to confer a significant survival benefit in both older and younger patients. To further improve on the treatment outcome of this disease, dose-dense, and/or dose-intense regimens have been developed and tested against CHOP. However, these regimens are not yet accepted as standard therapy because of the increased toxicity as well as the uncertain benefit over CHOP with rituximab. In patients with localized DLBCL, available randomized trials suggest that radiation therapy improves local control and disease-free survival and that the addition of radiation therapy cannot replace inadequate chemotherapy.     

Diffuse large B-cell lymphoma

Diffuse large B-cell lymphoma (DLBCL) remains a curable lymphoma, with improved outcome resulting in large part from the incorporation of rituximab in standard regimens. The disease is heterogeneous clinically, morphologically, and molecularly. Recent insights into the molecular heterogeneity of DLBCL are beginning to yield novel therapeutics with significant promise for key subsets of patients. Although cyclophosphamide, hydroxydaunorubicin, vincristine, and prednisone chemotherapy with rituximab remains a standard therapeutic approach for most patients who have DLBCL, it is anticipated that novel agents will be included in treatment regimens for many patients in the near future.     

Mechanisms of B-cell lymphoma pathogenesis

Chromosomal translocations involving the immunoglobulin loci are a hallmark of many types of B-cell lymphoma. Other factors, however, also have important roles in the pathogenesis of B-cell malignancies. Most B-cell lymphomas depend on the expression of a B-cell receptor (BCR) for survival, and in several B-cell malignancies antigen activation of lymphoma cells through BCR signalling seems to be an important factor for lymphoma pathogenesis. Recent insights into the lymphomagenic role of factors supplied by the microenvironment also offer new therapeutic strategies.     

Molecular markers of B-cell lymphoma

Molecular mapping of the cell surface has probably proceeded further with the human lymphocyte than with any other mammalian cell, and the B lymphocyte yields a wide range of subtly varying neoplasms. These two bodies of knowledge are now readily correlated, given the widespread adoption of a modern lineage-based classification of lymphoma (Revised European-American). Studies of the markers of B-cell lymphoma have immediate practical importance in diagnosis, defining clonality, and detecting minimal residual disease. They also help to keep us abreast of lymphocyte physiology, and present new opportunities for treating these neoplasms.     

Molecular features of B-cell lymphoma

Malignant transformation of B cells can occur at various steps of lymphocyte development, starting from early B-cell progenitors up to mature B cells, which reflects the heterogeneity of B-cell malignancies with regard to their biologic and clinical behavior. The genetic characterization of B-cell neoplasms during the past two decades has elucidated the mechanisms underlying B-cell lymphomagenesis and led to a more precise definition of lymphoma subgroups. This progress is reflected in the upcoming World Health Organization classification for hematologic neoplasms, which stresses the diagnostic importance of recurrent genetic alterations in leukemias and lymphomas. In the recent past, several genes deregulated by such recurrent chromosomal aberrations have been identified. In addition, the recent introduction of microarray technology has now allowed a more global assessment of gene dysregulation in B-cell oncogenesis and provided a new means for more exactly defining the molecular hallmarks of distinct lymphoma subtypes. This review will focus on recently described molecular features of B-cell lymphomas discovered by the application of new molecular cytogenetic techniques, advanced breakpoint cloning strategies, and microarray approaches.     

Primary mediastinal large B-cell lymphoma

Primary mediastinal large B-cell lymphoma represents a distinct entity with unique clinicopathologic features and a molecular gene-expression signature reminiscent of nodular sclerosis subtype of classical Hodgkin's lymphoma. Recent studies, including those using a refined molecular signature, suggest that the outcome is more favorable than that of diffuse large B-cell lymphoma. Using historical comparisons, dose-dense and dose-intensive regimens may be more effective than cyclophosphamide, doxorubicin, vincristine, and prednisone chemotherapy; however, the impact of adding rituximab to these regimens and effect on outcome comparisons is unknown. Clinical trials exploring these questions in addition to the benefit of consolidative radiotherapy are necessary to definitively answer these questions.