原发纵隔淋巴瘤研究进展：第19届欧洲血液学会年会报道

原发纵隔大B细胞淋巴瘤（PMBCL）是一种特殊类型的B细胞淋巴瘤,推测来源于胸腺髓质B细胞,占所有弥漫大B细胞淋巴瘤（DLBCL）的6％～10％,具有特殊的病理特征、免疫表型和基因异常,需要与经典型霍奇金淋巴瘤和其他结性DLBCL亚型鉴别.文章就PMBCL的病理学特征、分子生物学改变、治疗、预后及正电子发射型计算机断层扫描显像（PET）在评估初始治疗反应中的作用进行介绍.     

原发纵隔B细胞淋巴瘤研究进展

 【摘要】　新的WHO分类将原发纵隔B细胞淋巴瘤（PMBCL）归为弥漫大B细胞淋巴瘤（DLBCL）的一个独立亚型,其在发病机制、病理组织学、分子遗传学及临床特点等多个方面均与其他类型的DLBCL有所不同,与霍奇金淋巴瘤及纵隔灰区淋巴瘤又有着密切地联系,为加深临床医师对PMBCL的认识,就近年来的相关研究进行综述。     

弥漫大B细胞淋巴瘤预后相关因素研究进展

弥漫大B细胞淋巴瘤(DLBCL)是一种最常见的侵袭性非霍奇金B细胞淋巴瘤(B-NHL),在临床表现和预后等方面具有显著的异质性,多种因素影响其预后.文章根据国内外研究进展,从临床特征及分子生物学特征两方面对影响DLBCL患者预后的因素进行综述.     

颈部不一致性淋巴瘤一例并文献复习

目的 探讨不一致性淋巴瘤的临床病理学特征.方法 结合临床、影像学、病理形态和免疫组织化学检测,对1例不一致性颈部淋巴结结节硬化型霍奇金淋巴瘤及甲状腺左叶及峡部弥漫大B细胞淋巴瘤进行病理分析,并复习相关文献.结果 患者,女性,46岁,以甲状腺双叶实性肿物及双颈部多发淋巴结肿大为首发症状,手术切除甲状腺双叶及颈部淋巴结,术后病理诊断为不一致性淋巴瘤,右颈及部分左颈部淋巴结为经典型霍奇金淋巴瘤,甲状腺左叶及峡部为弥漫大B细胞淋巴瘤.结论 不一致性淋巴瘤较为罕见,表现形式多样,且发生的解剖部位不同,应引起临床和病理医师的足够重视,其治疗方案及预后判断由恶性程度高的肿瘤类型决定.     

原发纵隔大B细胞淋巴瘤的研究进展

原发纵隔大B细胞淋巴瘤（PMBCL）是一种原发于纵隔的侵袭性大B细胞淋巴瘤，由于其独特的临床和组织学特征，世界卫生组织淋巴肿瘤分类将PMBCL重新分类为一个单独的实体。PMBCL的诊断主要依赖于病理学特征、影像学检查和临床特征等。当前针对PMBCL的治疗方案有很多种，最常用的是R-CHOP和R-EPOCH方案等。放疗对部分患者有益，但也可以导致远期毒性。新药的研发也在不断进行，包括嵌合抗原受体T细胞疗法、抗程序性死亡受体1药物等，治疗后疗效判断和指导下一步的治疗策略主要依赖PET-CT。     

伴有二次打击的弥漫大B细胞淋巴瘤研究进展

弥漫大B细胞淋巴瘤（DLBCL）是一种异质性很大的非霍奇金淋巴瘤,其中同时伴有myc和bcl-2重排（或bcl-6重排）者是一种较少见的类型,被称作二次打击淋巴瘤.其临床表现为明显的侵袭性、对化疗耐药、预后极差,成为近年来研究的热点.文章探讨了伴有二次打击的DLBCL重排基因的功能、临床特征、诊断及治疗.     

原发纵隔的弥漫大B细胞非霍奇金淋巴瘤34例临床分析

目的:探讨原发纵隔的弥漫大B细胞非霍奇金淋巴瘤(PMLBL)的临床特征及治疗策略.方法:回顾性分析34例原发纵隔的弥漫大B细胞非霍奇金淋巴瘤的临床特点和不同治疗方案对患者生存期的影响.结果:临床分期79.4%为Ⅰ～Ⅱ期,50.0%患者起病时出现上腔静脉压迫综合征,47.1%有邻近器官侵犯.采用化、放疗联合治疗29例(85.3%)、单纯化疗5例(14.7%)、自体造血干细胞移植9例(26.4%).根据寿命表法分析,全组5和10年无瘤生存率(DFS)为47.8%和41.0%,5和10年总生存率(OS)为54.4%和54.4%.自体造血干细胞移植组较常规化、放疗组5年DFS和OS均有所提高,但无显著性差异(P＞0.05).不良预后因素分析显示,具有巨大肿块及治疗后未能获得完全缓解者预后差(P＜0.05).结论:原发纵隔的弥漫大B细胞非霍奇金淋巴瘤具有独特的临床和病理特点,治疗以化、放疗为主.具有不良预后因素的患者可考虑自体外周血造血干细胞移植(APBSCT),但最佳的治疗方案尚需进一步的前瞻性随机研究证实.     

侵袭性B细胞淋巴瘤中PIK3CA扩增和PTEN缺失及其临床病理学意义

  目的  探讨侵袭性B细胞淋巴瘤在PI3K/Akt/mTOR信号通路中PIK3CA与10号染色体上缺失的磷酸酶和张力蛋白同源物（phosphate and tension homology deleted on chromsome ten,PTEN）的表达情况及其与各临床病理指标的相关性。  方法  回顾性分析新疆医科大学第一附属医院2008年1月至2012年12月术前未经任何治疗的侵袭性B细胞淋巴瘤标本235例,其中包括弥漫大B细胞淋巴瘤（diffuse large B-cell lymphoma,DLBCL）205例,伯基特淋巴瘤（Burkitt lymphoma,BL）27例,灰区淋巴瘤（介于DLBCL和BL之间,不能分类的B细胞淋巴瘤）3例。应用荧光原位杂交技术检测所有样本PIK3CA和PTEN基因的表达情况,统计分析其与各临床病理指标及预后的关系。  结果  PIK3CA在侵袭性B细胞淋巴瘤中扩增率为12.3%（29/235）,临床分期Ⅰ~Ⅱ期和Ⅲ~Ⅳ期的阳性率分别为8.6%（12/139）和17.7%（17/96）,差异具有统计学意义（P=0.038）。PTEN在侵袭性B细胞淋巴瘤中的缺失率为13.6%（32/235）,与其他临床病理特征无相关性。PIK3CA扩增和PTEN缺失呈负相关（P=0.046）。未发现PIK3CA扩增和PTEN缺失与生存期存在相关性。  结论  PIK3CA扩增与侵袭性B细胞淋巴瘤疾病晚期相关,PIK3CA扩增和PTEN缺失在侵袭性B细胞淋巴瘤的发生中起促进作用。      

microRNA与弥漫大B 细胞淋巴瘤关系的研究进展

微小RNA（microRNA,miRNA）是一类长度为20多个核苷酸的内源性非编码调控RNA。它们通过序列特异性翻译抑制或mRNA 裂解来调控基因表达,参与细胞发育、增殖、分化、凋亡等一系列重要生物学过程。近期的研究发现,许多microRNA具有癌基因或抑癌基因的作用,在肿瘤的发生和发展中起着重要的作用。弥漫大B 细胞淋巴瘤（DLBCL）是最常见的一类恶性淋巴瘤,占非霍奇金淋巴瘤的30% 左右,DLBCL被定义为B 细胞起源的、有大的肿瘤细胞、具有侵袭性临床表现、需要高效力的化学治疗的恶性淋巴瘤,这类肿瘤发生于淋巴结内或结外,可原发或继发于其它低度恶性淋巴瘤的演进,临床表现、形态学、免疫表型及遗传学特征极具异质性。现已发现若干种microRNA直接参与弥漫大B 细胞淋巴瘤的发生和发展;microRNA表达谱也与弥漫大B 细胞淋巴瘤分子亚型相关。作为一类新的分子靶标,microRNA被应用于弥漫大B 细胞淋巴瘤的诊断和生物治疗具有广阔的前景。      

美罗华联合化疗治疗老年人难治和复发性弥漫性大B细胞淋巴瘤的临床研究

弥漫性大B细胞型淋巴瘤是非霍奇金淋巴瘤(non-Hodgkin's lymphomas,NHL)中最常见的亚型,其生物学行为属侵袭性.CHOP方案是治疗侵袭性B细胞型非霍奇金淋巴瘤的标准方案.     

Primary Mediastinal Large B-Cell Lymphoma, Classic Hodgkin Lymphoma Presenting in the Mediastinum, and Mediastinal Gray Zone Lymphoma: What is the Oncologist To Do?

In recent years, an overlap in biologic and clinical features has been identified between classic Hodgkin lymphoma (CHL) and primary mediastinal large B-cell lymphoma (PMBL). Further strengthening this relationship is the identification of lymphomas with clinical and morphologic features transitional between the two, known as gray zone lymphomas (GZL). However, this diagnostic gray zone is not just of theoretical interest: it presents a practical problem, as the treatment approaches for CHL traditionally differ from those for aggressive B-cell lymphomas. This article reviews the treatment approach for mediastinal lymphomas, including CHL of the nodular sclerosis subtype (CHL-NS), PMBL, and mediastinal GZL. Though several trials have evaluated different regimens with or without radiation in PMBL and CHL-NS, there is a lack of prospective experience in treating GZL because of the rarity of these tumors. Historical data indicate that they have done poorly with traditional approaches developed for the treatment of either CHL or diffuse large B-cell lymphoma.     

U-2940, a human B-cell line derived from a diffuse large cell lymphoma sequential to Hodgkin lymphoma

Several patterns of association between Hodgkin and non-Hodgkin lymphomas are recognized, some of which support a common cellular origin or shared transformation events for both malignancies. We describe the U-2940 cell line derived from a diffuse large B-cell lymphoma with some features consistent with mediastinal large B-cell lymphoma, clinically apparent 1 month after the initial course of chemotherapy for Hodgkin's disease, fulfilling the criteria for composite malignancies. U-2940 cells display a mature B phenotype with hypermutated IgH rearrangement typical of germinal/postgerminal center origin. The cell line is negative for Epstein-Barr virus and no evidence of t(14;18) was found. U-2940 cells display multiple chromosomal rearrangements similar to recurrent aberrations described in both Hodgkin and non-Hodgkin lymphomas, also partially shared by U-2932 derived from a B-cell lymphoma sequential to Hodgkin's disease. The original large B-cell lymphoma and the U-2940 cell line bear microsatellite instability, an abnormality associated with particular subtypes of non-Hodgkin lymphomas and found in tissues involved by Hodgkin lymphoma. Therefore, U-2940 cells bear several features known to occur in Hodgkin and in non-Hodgkin lymphomas, leading to the assumption that this cell line may constitute a useful tool to address elective pathways of lymphomagenesis and eventually the Hodgkin and non-Hodgkin lymphoma association.     

Gray Zone Lymphoma: Better Treated Like Hodgkin Lymphoma or Mediastinal Large B-Cell Lymphoma?

Although primary mediastinal large B-cell lymphoma (PMBL) and classic Hodgkin lymphoma of the nodular sclerosis type (CHL-NS) are distinct diseases, they share several clinical characteristics and biologic features. Given that, it is not surprising that there exist mediastinal lymphomas that do not fit well into either category but have clinical and morphologic features overlapping and transitional between PMBL and CHL-NS. The term mediastinal gray zone lymphoma (MGZL) has been used for these tumors, which are included in the World Health Organization classification as "B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and classic Hodgkin lymphoma." Although several studies have evaluated different therapeutic strategies in PMBL and CHL-NS, there is a paucity of prospective experience treating MGZL, given its rarity and relatively recent recognition. Historically, diseases that today would be categorized as MGZL were probably called "anaplastic large-cell lymphoma Hodgkin-like," and their outcome with standard approaches was poor, with short overall survivals. In this review-following a discussion of the biology and clinical features of MGZL, and how they compare to PMBL and CHL-NS-we outline how the treatment of PMBL and CHL-NS has evolved in recent years, and how we believe MGZL should be approached therapeutically.     

儿童侵袭性成熟B细胞淋巴瘤的临床病理学及分子遗传学特点

 目的　分析总结中国儿童各类型侵袭性成熟B细胞淋巴瘤的临床病理学及分子遗传学特点,为其诊断的标准化提供依据。方法　收集97例儿童侵袭性成熟B细胞淋巴瘤石蜡包埋组织标本,包括伯基特淋巴瘤（BL）81例、弥漫大B 细胞淋巴瘤（DLBCL）8例、介于BL和DLBCL间的不能分类的B细胞淋巴瘤（BL／DLBCL）8例,利用免疫组织化学技术和间期荧光原位杂交（FISH）技术检测其免疫表型和分子遗传学特征。结果　BL的bcl-2和MUM1的阳性率分别为3 %（2／66）和17 %（12／71）,DLBCL分别为50 %（4／8）和63 %（5／8）,BL／DLBCL分别为 50 %（4／8）和63 %（5／8）。BL、DLBCL 和BL／DLBCL 的Ki-67平均值分别为（93±4.4）%、（83±14.3）%和（80±11.5）%。BL、DLBCL 和BL／DLBCL 的c-myc 基因易位的比例分别为98 %（79／81）、38 %（3／8）和50 %（4／8）。38 %（3／8）的DLBCL和25 %（2／8）的BL／DLBCL 存在bcl-6基因的多拷贝,BL与DLBCL 之间、BL与BL／DLBCL之间bcl-2、MUM1和 Ki-67平均值的差异及c-myc基因易位和bcl-6基因多拷贝的差异均有统计学意义（均P＜0.05）。结论　儿童侵袭性成熟B细胞淋巴瘤的诊断和分型需要综合分析形态学、免疫表型和分子遗传学特征。儿童BL／DLBCL 可能是DLBCL 的一个亚型。CD+10、bcl-6+、bcl-2-、Ki-67＞90 %、伴有IGH／c-myc重排、不伴有bcl-2和bcl-6重排时,支持BL的诊断;bcl-2+、Ki-67 为50 %～90 %,同时伴有bcl-6基因的多拷贝时,支持 DLBCL或BL／DLBCL 的诊断。     

原发性肺淋巴瘤11例临床特点分析

目的探讨原发性肺淋巴瘤的发病情况、临床特点、影像学特点、治疗及预后情况。方法回顾性分析了11例原发性肺淋巴瘤的临床资料及随访情况。结果11例患者中,霍奇金淋巴瘤2例,非霍奇金淋巴瘤9例（B细胞性4例,T细胞性4例,未分型1例）。临床症状多表现为咳嗽,咳痰,胸背部疼痛。影像学多表现为肺部肿块,边缘不规则,增强可强化,部分伴有肺不张。9例非霍奇金淋巴瘤患者中6例化疗的同时接受了放疗,3例仅接受了化疗,2例霍奇金淋巴瘤只接受了化疗。除了1名患者因各种因素造成脱落随访外,剩余10例随访患者的总生存期为15～102个月（中位生存期为84．5个月）。结论肺内淋巴瘤患者临床表、影像学检查无特异性．易误诊．治疗以化疗为主．辅以放疗．     

Cyclin D1和bcl-2在B细胞淋巴瘤中的表达及其在鉴别诊断中的意义

 B细胞淋巴瘤根据免疫表型可分为不同亚型,且不同亚型侵袭度不同,预后也有很大差异。Cyclin D1是已被证实与肿瘤有最直接关系的细胞周期蛋白,在大多B细胞淋巴瘤[套细胞淋巴瘤（MCL）、慢性淋巴细胞白血病（CLL）、边缘区淋巴瘤（MZL）、弥漫大B细胞淋巴瘤（DLBCL）等]中均有表达。多数B细胞淋巴瘤[滤泡性淋巴瘤（FL）、DLBCL等]都能可见易位活化的bcl-2表达增强。Cyclin D1及bcl-2作为B细胞淋巴瘤重要的细胞周期蛋白及抗凋亡基因,在淋巴瘤的鉴别诊断中起重要作用,其检测及检测手段的灵敏度和特异度具有重要的临床价值。     

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.