继发性B细胞型非霍奇金淋巴瘤患者的临床特征分析

目的　探讨继发于非血液系统恶性肿瘤的B细胞型非霍奇金淋巴瘤（sNHL）患者的临床特征及预后情况。方法　2007年1月—2018年5月就诊于我院的NHL患者中,1.65%（47/2 853）以第二恶性肿瘤的形式出现,其中44例为B细胞型sNHL。44例中第一原发恶性肿瘤治疗情况：29例为手术切除治疗,10例为手术联合化疗,2例为手术联合放疗,3例接受手术联合化疗及放疗。继发肿瘤的治疗情况：34例患者接受化疗,5例患者接受化疗+手术切除,4例患者因结外病变或巨大包块接受放疗,1例结外边缘区B细胞淋巴瘤,黏膜相关淋巴组织型淋巴瘤（胃型）患者仅接受抗幽门螺杆菌治疗。中位化疗时间为6（2,8）个月。CD20+的弥漫大B细胞淋巴瘤（DLBCL）或CD20+Ⅲ～Ⅳ期滤泡淋巴瘤（FL）患者在化疗前1 d静脉滴注利妥昔单抗（375 mg/m2）。中位随访时间11.4（4.2,28.8）个月。结果　44例sNHL患者最常见的第一原发恶性肿瘤疾病类型为乳腺癌（10例）,最常见的sNHL病理类型为DLBCL（29例）。65.9%（29/44）的患者第一原发肿瘤诊断年龄＜60岁,59.1%（26/44）的患者sNHL诊断年龄≥60岁。中位sNHL发生时间为63.4（25.2,146.9）个月。平均无疾病进展时间为9.6（4.1,26.0）个月。3年总生存率为73.5%。一线治疗后完全缓解率为38.6%,总缓解率为63.6%。利妥昔单抗组与未利用利妥昔单抗组的3年总生存率分别为81.1%和66.5%（Log-rank χ2=2.026,P＞0.05）。单因素分析显示第一原发恶性肿瘤诊断年龄≥60岁（Log-rank χ2=7.562,P＜0.05）,sNHL诊断年龄≥60岁（Log-rank χ2=4.887,P＜0.05）均提示预后不良;多因素分析显示第一原发恶性肿瘤发病年龄≥60岁（HR=4.745,95%CI：1.405～16.020）是影响sNHL患者生存率的独立危险因素。结论　第一原发恶性肿瘤的诊断年龄≥60岁会增加sNHL患者的死亡风险。     

1例非霍奇金淋巴瘤双侧肾上腺、心脏等多脏器浸润报道

非霍奇金淋巴瘤（NHL）是起源于淋巴结和淋巴组织的恶性肿瘤,其发生与病毒感染及免疫功能不全有关。NHL有多中心起源,而弥漫大B细胞淋巴瘤（DLBCL）是NHL的一种,多数表现为侵袭性,容易早期远处转移及结外侵犯倾向;常可侵犯肝脾、眼部、肺、胃肠、骨骼、泌尿生殖系统等脏器,但累及心脏、肾上腺相对少见。最近在临床遇到1例DLBCL结外侵犯多个内脏器官的病例,报道如下。     

原发性甲状腺淋巴瘤的诊断和治疗进展

原发性甲状腺淋巴瘤(primary thyroid lymphoma,PTL)是罕见的甲状腺恶性肿瘤,其中弥漫性大B细胞淋巴瘤(DLBCL)和低度恶性黏膜相关淋巴组织淋巴瘤(MALT)为最常见的两种病理类型.PTL发病与甲状腺的慢性炎症刺激和异常的体细胞高度突变相关.PTL术前诊断较困难,治疗方式复杂多变.本文阐述PTL的影像学、病理学诊断和治疗新进展.     

心脏弥散性大B细胞淋巴瘤行心脏移植术后复发1例的护理

弥散性大B细胞淋巴瘤（diffuse large B cell lymphoma,DLBCL）是成人淋巴瘤中最常见的类型,是一组在临床表现、组织形态和预后等多方面具有很大异质性的恶性肿瘤,临床以迅速增大的无痛性肿块为典型表现。     

十二指肠原发性恶性淋巴瘤8例分析

二十指肠原发性恶性淋巴瘤是淋巴结以外的淋巴组织的恶性肿瘤,临床上较少见,由于本病病史、症状及体征缺乏特异性,所以临床上极易误诊,由于恶性淋巴瘤与假性淋巴瘤（良性增生病变）有最本质的区别,并且恶性淋巴瘤与其它恶性肿瘤在发病机理、治疗及预后等方面也存在差异,所以从病理学角度把它们加以鉴别有十分重要意义。为此,我们用HE染色、免疫组化,结合临床资料对8例十二指肠原发性恶性淋巴瘤的病理诊断特征进行分析。     

弥漫性大B细胞淋巴瘤中CD40L的表达及意义

目的探讨弥漫性大B细胞淋巴瘤（DLBCL）组织中CD40L表达与DLBCL预后间的关系及意义。方法免疫组织化学法检测27例弥漫性大B细胞淋巴瘤、20例淋巴结反应性增生组织中CD40L的表达。结果（1）DLBCL中CD40L过度阳性率（25．93％）显著低于淋巴结反应性增生（63．64％），P〈0．05。（2）CD40L在Ⅲ、Ⅳ期DLBCL过度阳性率（14．29％）低于Ⅰ、Ⅱ期（38．46％），P〈0．05。CD40L过度阳性率在有结外浸润DLBCL（11．76％）低于无有结外浸润DLBCL（40％），P〈0．05。（3）DLBCL患者CD40L的过度阳性率与远处转移、临床分期均显著相关，P〈0．05。结论（1）CD40L过度阳性率与结外器官浸润及临床分期密切相关，其可能作为判断DLBCL侵袭性及预后的指标。（2）DLBCL中CD40L表达的减少可能是影响其发病的因素之一。     

原发性中枢神经系统淋巴瘤32例临床分析

目的 探讨原发性中枢神经系统淋巴瘤（PCNSL）临床特点、诊治方案及临床疗效。方法 回顾性分析1990年1月～2004年12月收治的PCNSL32例。结果 1例经立体定向活检确诊,31例手术确诊。31例手术中全切或近全切除28例（90％）,大部切除3例（10％）,无手术死亡病例。病理检查：B细胞型淋巴瘤30例,T细胞型淋巴瘤2例。术后临床症状改善22例（69%）,稳定6例（19％）,症状加重4例（12％）。术后辅以放射治疗25例,联合化疗9例。结论 PCNSL为少见恶性肿瘤,早期诊断,并进行有效的综合治疗是延长PCNSL患者生存期和改善生存质量的关键。     

肝硬化合并以上消化道大出血为首发症状的胃弥漫大B细胞淋巴瘤一例

弥漫大B细胞淋巴瘤（DLBCL）是非霍奇金淋巴瘤（NHL）中发病率最高的一类弥漫生长的B细胞淋巴瘤。该病以进行性淋巴结肿大为最主要的临床表现,可结外发病,临床表现多样性,易被误诊。本文报告为以上消化道出血为首发症状,合并肝硬化,内镜表现为胃溃疡的胃DLBCL患者1例。期望通过对本病例分析以提高对DLBCL的认识和诊治水平。     

基于GEO数据库分析源于滤泡性淋巴瘤的转化性弥漫大B细胞淋巴瘤的基因表达特点

目的 筛选出来自于滤泡性淋巴瘤的转化性弥漫大B细胞淋巴瘤（transforming follicular lymphoma,tFL）与滤泡性淋巴瘤（follicular lymphoma,FL）和弥漫大B细胞淋巴瘤（diffuse large B cell lymphoma,DLBCL）的差异表达基因并进行生物信息学分析，从而探究它们间的差异以及tFL发展的生物学过程，为后续实验寻找潜在靶点。方法 利用GEO数据库筛选出t FL、FL、DLBCL的基因集，分析差异基因，进行GEO富集可视化分析、基因本体（Gene ontology,GO）富集分析、京都基因和基因组数据库（Kyoto Encyclopedia of Genes and Genomes）通路分析。结果 tFL与FL的基因集共找到11 388个差异表达基因，GO富集分析发现差异基因的功能主要集中在轴突生成、树突发育等；KEGG通路分析发现关键途径为轴突导向、Hippo信号通路等。而tFL与DLBCL共有17216个差异表达基因，GO富集分析差异基因的功能主要集中在调节细胞发育、组蛋白的修饰等；KEGG通路分析发现关键途径为轴...     

原发性鼻腔非霍奇金淋巴瘤1例

近年来,由于医学技术的不断发展,发现了一些过去比较少见的鼻腔恶性肿瘤疾病病例。其中,鼻腔非霍奇金淋巴瘤（NasalNon—Hodgkinlym--phoma,N-NHL）的报道有所增多。我科于2011年6月收治了1例鼻腔非霍奇金弥漫性大B细胞淋巴瘤,现报道如下。     

Treatment strategies for relapsed and refractory aggressive non-Hodgkin's lymphoma

The aggressive non-Hodgkin's lymphomas (NHL) are a clinically heterogeneous group of lymphomas with disparate responses to standard chemotherapy regimens. Aggressive NHL includes diffuse large B-cell lymphoma (DLBCL), mantle cell lymphoma (MCL) and peripheral T-cell lymphomas (PTCL), among others. Significant advances have been made in the last decade in the initial treatment of DLBCL and MCL, but the treatment of relapsed or refractory disease remains difficult. The addition of rituximab to the treatment of DLBCL and MCL has improved clinical outcomes and is now a critical component of initial therapy and treatment of relapsed disease. The PTCLs, not having a similar agent to significantly change the treatment approach to these diseases, remain a difficult therapeutic problem. This review examines recent advances in the treatment of relapsed or refractory aggressive NHL and discusses novel approaches currently under investigation.     

CD56在非霍奇金淋巴瘤中的表达和意义

目的探讨CD56在非霍奇金淋巴瘤中NK/T细胞淋巴瘤的表达和意义。方法应用组织形态学及免疫组织化学分析对29例我院既往诊断的恶性淋巴瘤组织进行HE、PSA染色,同时作CD56的免疫表型分析。结果10例弥漫性大B淋巴瘤和6例外周T细胞淋巴瘤肿瘤细胞CD56表达均阴性,12例NK/T细胞淋巴瘤CD56表达阳性,1例NK/T细胞淋巴瘤可疑。结论CD56可作为非霍奇金淋巴瘤中NK/T细胞淋巴瘤分类鉴别的重要指标。     

弥漫大B细胞淋巴瘤治疗的研究进展

弥漫大B细胞淋巴瘤（DLBCL）属于侵袭性B细胞淋巴瘤,目前利妥昔单抗联合环磷酰胺+蒽环类+长春碱类+糖皮质激素（R-CHOP方案）已成为治疗DLBCL的一线治疗方案,但部分患者存在复发或疾病进展情况,针对这类患者的治疗仍是目前临床研究关注的重点。目前针对复发/难治DLBCL患者的治疗主要有抗体药物偶联物、靶向药物、免疫检查点抑制剂、细胞免疫治疗及造血干细胞移植等,有望为患者的疾病预后带来显著改善。     

Pharmacotherapy of large B-cell lymphoma

Background: Constituting approximately 30% of lymphoid malignancies, diffuse large B-cell lymphoma (DLBCL) is the most common aggressive lymphoma in adults worldwide. The clinical and biologic heterogeneity that exists in DLBCL suggests that this entity might actually be comprised of several distinct neoplasms that could require different therapeutic approaches. DLBCL was considered incurable until combination chemotherapy became available. Objective: Current treatment strategies for the treatment of untreated and relapsed advanced-stage DLBCL are reviewed; novel treatments for DLBCL are discussed. Methods: Relevant literature was identified using the PubMed search engine and by reviewing abstracts from major conference proceedings. Results/conclusion: Recently, novel therapeutic strategies, including the incorporation of immunotherapy to combination chemotherapy, have improved outcome for patients with DLBCL with cure rates exceeding 50%, especially in younger patients.     

弥漫大B 细胞淋巴瘤研究进展

弥漫大B细胞淋巴瘤(DLBCL)是成人最常见的非霍奇金B细胞淋巴瘤,在第四版WHO造血与淋巴组织肿瘤分类中,根据形态学及临床特征,将DLBCL分为非特殊类型、特殊亚型和独立疾病。DLBCL在形态学、免疫表型、遗传学特征及临床表现上具有显著的异质性。根据基因表达谱,DLBCL可分为预后较好的生发中心B细胞型(GCB型)DLBCL和活化B细胞型(ABC型)DLBCL。根据免疫组化分型,也可将DLBCL分为GCB型和非GCB型(或ABC型)。GCB型和非GCB型(或ABC型)的预后因素通过不同的通路而发挥其生物学作用,表现在蛋白的异常表达或基因的断裂重排、扩增及易位。但这些蛋效应是否影响着应用利妥昔单抗治疗的DLBCL患者的预后,目前还不是很确定。本文通过对DLBCL的分类、免疫分型等研究进展的总结,增强对该疾病的认识。     

Enzastaurin hydrochloride for lymphoma: reassessing the results of clinical trials in light of recent advances in the biology of B-cell malignancies

Introduction: The B-cell receptor (BCR) is critical for the development and persistence of B-cell non-Hodgkin lymphoma (B-NHL). Protein kinase C-beta (PKC-β) has been identified as one of the key signaling hubs downstream of the BCR and constitutes a valuable target in B-NHL. As a potent PKC-β inhibitor, enzastaurin is currently being tested in Phase II/III trials.

Areas covered: This review summarizes the latest results and ongoing clinical trials with enzastaurin in light of basic scientific advances in the understanding of various lymphoid cancers, including diffuse large B-cell lymphoma (DLBCL), mantle cell lymphoma (MCL), follicular lymphoma (FL), chronic lymphocytic leukemia (CLL) and Waldenström's macroglobulinemia (WM).

Expert opinion: While its continued clinical development is uncertain, enzastaurin should be regarded as a stepping stone for the development of future therapies; indeed, the recent research has provided valuable insight into the possible molecular mechanisms that explain its limited clinical activity especially in the treatment of DLBCL and MCL. It should be noted that there is still some interest in enzastaurin, in combination, for the treatment of WM.     

Lenalidomide for the treatment of B-cell lymphoma

Introduction: Although the combination of an anti-CD20 monoclonal antibody and chemotherapy has widely improved survival of patients with B-cell lymphoma, the disease still relapses. A better understanding of the biology of lymphomas has highlighted the role of the cell of origin in response to treatment and outcome. Lenalidomide represents an attractive therapeutic option due to its original mechanism of action.

Areas covered: In this review, the authors describe the pharmacological properties of lenalidomide, and the rational for its use in B-cell lymphomas; focusing on diffuse large B-cell lymphoma (DLBCL), follicular lymphoma (FL), and mantle cell lymphoma (MCL). They discuss the mechanism of action of the drug and its current and future clinical development. They also review the current data in relapsed/refractory situations as well as in first-line treatment.

Expert opinion: Lenalidomide is an oral non-chemotherapy immunomodulatory agent with an acceptable toxicity profile and manageable side-effects. Efficacy has widely been demonstrated, especially in MCL, FL and non-Germinal Center DLBCL patients. Further studies are now warranted to better define the strategy for the use of lenalidomide in B-NHL patients, and clarify which subgroup of patients will really benefit of lenalidomide as part of first-line treatment or in a relapsed/refractory setting.