弥漫大B细胞淋巴瘤免疫表型分型与预后的关系

目的 探讨弥漫大B细胞淋巴瘤（DLBCL）的免疫表型之生发中心B细胞样（GCB）和非GCB两个亚型的特征及其与DLBCL预后的关系。方法 根据肿瘤细胞免疫组织化学EnVision法标记CD10、bc1-6、MUM-1的表达情况,将133例DLBCL分为GCB和非GCB两个亚型。对以下指标的5年总生存率（OS）及5年无进展生存率（PFS）进行了比较：（1）CD10、bc1-6和MUM-1的阳性和阴性病例;（2）GCB亚型与非GCB亚型;（3）不同国际预后指数（IPI）分组中GCB亚型与非GCB亚型的关系。结果 133例DLBCL中,44例（33．1％）CD10阳性,48例（34．6％）bc1-6阳性,60例（45．1％）MUM-1阳性。CD10阳性DLBCL患者的5年OS及PFS均明显高于CD10阴性患者（P=0．041和0．031）;bc1-6阳性DLBCL患者的PFS明显高于bc1-6阴性患者（P=0．044）,MUM．1阳性DLBCL患者的5年0s及PFS均明显低于MUM-1阴性患者（P=0．031和0．028）。GCB型54例（40．6％）,非GCB型79例（59．4％）。GCB型5年OS及PFS均明显高于非GCB型（P=0．004和0．003）。国际预后指数（IPI）0-1分组及2-5分组中,GCB型5年OS及PFS均明显高于非GCB型（IP10-1分组P=0．019和0．014,2-5分组P=0．006和0．009）,其中IPI2-5分组中的非GCB预后最差。结论 DLBCL亚型及其与IPI联合分析可以作为预测患者预后的有效指标。     

弥漫性大B细胞淋巴瘤免疫分型与分子遗传学异常的研究

目的 分析弥漫性大B细胞淋巴瘤(diffuse large B-cell lymphoma,DLBCL)的免疫表型分型和分子遗传学异常,并探讨二者之间的相关性及其对于预后的意义.方法 用免疫组织化学链霉亲和素-过氧化物酶法检测59例DLBCL患者中CD10、BCL6、MUM1的表达,采用Hans免疫分型法将DLBCL分为生发中心B细胞(germinal center B-cell,GCB)型和非GCB型;分别应用BCL6双色断裂点分离探针、IgH/BCL2双色双融合探针及MYC双色断裂点分离探针在石蜡包埋的淋巴瘤组织切片上进行荧光原位杂交分析,检测BCL6、BCL2和MYC基因的易位和扩增情况.结果 在59例DLBCL患者中,GCB型占28.8％(17/59),非GCB型占71.2％ (42/59).BCL6、BCL2和MYC基因易位的发生率分别为24.1％(14/58)、1.7％(1/59)和5.3％(3/57),BCL6、BCL2和MYC基因扩增的发生率分别为17.2％ (10/58)、22.0％(13/59)和21.1％(12/57).BCL6扩增与BCL6易位无相关性(P=0.424),但与BCL2及MYC扩增呈正相关(列联系数C值分别为0.405和0.403,P值均＜0.01).在GCB型中BCL6易位的发生率高于非GCB型,而BCL6、BCL2或MYC扩增更常见于非GCB型,差异接近但均无统计学意义(P值分别为0.089和0.106).在单因素分析中,免疫分型和国际预后指数积分对总生存率(overall survival,OS)均有显著影响(P值分别为0.047和0.001),而BCL6易位和3基因扩增对OS率均无明显影响(P值分别为0.150和0.444);在多因素分析中,国际预后指数积分是影响OS唯一的独立预后因素(RR=3.843,P=0.017).结论 本组DLBCL患者中GCB亚型较少见,常见的遗传学异常为BCL6易位和BCL6、BCL2及MYC扩增,且3基因扩增之间密切相关,而BCL2易位的发生率低.免疫表型分型对预后的预测意义较小,遗传学异常不能用于预测DLBCL患者的预后.     

儿童腹腔非霍奇金B细胞淋巴瘤的临床病理及免疫表型分析

目的 探讨儿童腹腔原发性非霍奇金B细胞淋巴瘤的临床病理、免疫表型与EBER特征及其病理诊断和鉴别诊断.方法 按WHO(2008年)淋巴瘤分类标准分析74例儿童腹腔原发性非霍奇金B细胞淋巴瘤的临床病理资料,制备组织芯片,进行免疫组织化学SP法染色,EBER原位杂交和c-myc基因荧光原位杂交,观察CD20、CD79a、CD3、CD10、bcl-6、MUM1、bcl-2、CD43、CD38和Ki-67蛋白的表达和EBER表达特征,并区分伯基特淋巴瘤(BL)、弥漫性大B细胞淋巴瘤(DLBCL)和介于BL和DLBCL之间的不能分类的B细胞淋巴瘤(DLBCL/BL)病理类型,在DLBCL中再区分其生发中心B细胞型(GCB)和非生发中心B细胞型(non-GCB)的分化特征.结果 儿童腹腔非霍奇金B细胞淋巴瘤中BL为65例(87.8%),DLBCL为4例(5.4%),DLBCL/BL为5例(6.8%).临床以腹痛、腹部包块、肠梗阻及肠套叠为主要发病症状.BL免疫组织化学表达CD20(65例)、CD79a(65例)、CD10(63例)、bcl-6(62例)、MUM1(15例)、CD43(46例)和CD38(63例);不表达CD3、bcl-2;27例(41.6%)EBER阳性;54例(93.0%)c-myc基因位点断裂.DLBCL免疫组织化学表达CD20(4例)、CD79a(4例)、CD10(3例)、bcl-6(2例)、MUM1(2例)、bcl-2(3例)、CD43(2例)、CD38(2例);不表达CD3;其中2例GCB,2例non-GCB;EBER阴性;1例c-myc基因位点断裂.DLBCL/BL免疫组织化学表达CD20(5例)、CD79a(5例)、CD10(5例)、bcl-6(4例)、MUM1(3例)、CD43(5例)、CD38(3例),不表达CD3和bcl-2;4例EBER阴性;3例c-myc基因位点断裂.结论 儿童腹腔非霍奇金B细胞淋巴瘤具有侵袭性生长的特点,以BL为主要病理类型.临床以腹痛、腹部包块、肠梗阻及肠套叠为主要发病症状,主要累及回盲部肠组织及周围系膜淋巴结,病理形态、免疫表型、EBER、c-myc基因的检测对BL、DLBC及DLBCL/BL淋巴瘤的诊断和鉴别诊断有重要作用.     

儿童弥漫大B细胞淋巴瘤的临床病理、免疫亚型及C-MYC基因分析

弥漫大B细胞淋巴瘤(diffuse large B cell lymphoma,DLBCL)是由弥漫增生的大B淋巴样细胞构成的成人最常见的一种非霍奇金淋巴瘤(non-Hodgkin's lymphoma,NHL),在儿童较少见,国外报道占儿童NHL的10％[1],国内目前尚未见儿童DLBCL发病率的报道.本研究报道6例儿童DLBCL,并结合文献探讨其临床病理特点、免疫亚型及C-MYC基因的特征.     

原发胃肠道弥漫性大B细胞淋巴瘤的免疫分型及其与预后的关系

目的 了解原发胃肠道弥漫性大B细胞淋巴瘤的免疫分型,并比较Choi、Tally和Hans 分型及其与预后的关系.方法 复习90例原发胃肠道弥漫性大B细胞淋巴瘤患者的临床及病理资料并进行随访,应用Kaplan-Meier法、Log-rank检验和Cox比例风险回归模型对临床资料、实验室检测结果 进行生存分析及单因素和多因素预后分析.免疫表型检测采用EnVision和EliVision法,选用的抗体有CD20、CD3ε、CDl0、bcl-6、MUM-1、CD5、bcl-2、GCET1、FOXP1、LMO2、BLIMP1和Ki-67等.结果 (1)年龄为27～83岁,中位年龄58岁,男女比为1.31:1;胃肿瘤58例,占64.4%(58/90);肠肿瘤32例,占35.6%(32/90).(2)肿瘤细胞均表达CD20抗原,均不表达CD3ε和CD5;CD10、bcl-6、MUM-1(30%/80%阈值)的表达率分别为17.8%(16/90)、75.6%(68/90)、52.2%(47/90)/43.3%(39/90),GCET1、FOXP1、LMO2的表达率分别为50.0%(45/90)、45.6%(41/90)、23.3%(21/90),bcl-2、BLIMP1的表达率分别为42.2%(38/90)、8.9%(8/90),Ki-67阳性指数20%～95%,中位数为80%.Hans分型:51.1%为生发中心B细胞型(GCB型),48.9%为非GCB型;Choi分型:55.6%为GCB型,44.4%为活化B细胞(ABC)型;Tally分型:34.4%为GCB型,65.6%为非GCB型.(3)67.8%(61/90)的患者接受化疗,68.9%(62/90)的患者接受手术.患者的2、3和5年总体生存率分别为58.5%、52.8%和49.8%,CHOP方案(环磷酰胺+多柔比星+长春新碱+泼尼松)治疗组的2、3和5年总体生存率分别为68.5%、61.2%和52.9%.结论 Hans和Choi分型各亚型比例差别不大,Tally分型中非GCB型较GCB型比例增高.三种分型的各亚型均存在GCB型优于非GCB/ABC型的趋势.Log-rank检验单因素分析提示乳酸脱氢酶(LDH)水平、国际预后指数(IPI)、化疗、手术、B症状、病变数量、临床分期对预后有影响.Cox比例风险回归模型多因素分析提示Hans分型、Choi分型、化疗、手术、LDH和Lugano分期是独立的预后因素.

Abstract：

Objective To study the immunophenotype and prognostic significance of primary gastrointestinal diffuse large B-cell Iymphoma, with reference to Hans, Choi and Tally algorithms. Methods The clinicopathologic features and follow-up data in 90 cases of primary gastrointestinal diffuse large B-cell lymphoma were analyzed by Kaplan-Meier method, Log-rank test and Cox regression model.Immunohistochemistry was carried out using EliVision and EnVision methods for CD20, CD3ε, CD10,bcl-6, MUM-1, CDS, bcl-2, GCET1, FOXP1, LMO2,BLIMP1 and Ki-67. Results The age of patients studied, 64. 4% (58/90) involved the stomach and 35.6% (32/90) involved the intestine. The immunohistochemical findings were as follows: 100% positivity for CD20, 0% for CD3ε and CD5, 17.8% (16/90) for CD10, 75.6% (68/90) for bcl-6, 52. 2% (47/90) for MUM-1 (cut off was 30%), 43.3%(39/90) for MUM-1 (cut off was 80%), 50.0% (45/90) for GCET1, 45.6% (41/90) for FOXP1,23.3%(21/90) for LMO2, 42.2% (38/90) for bcl-2 and 8.9% (8/90) for BLIMP1. The Ki-67 index ranged from 20% to95% (median =80%). According to Hans algorithm, 51.1% of the cases belonged to germinal center B-cell (GCB) subtype and 48.9% belonged to non-GCB subtype. In contrast, Choi algorithm classified 55.6% cases as GCB subtype and 44. 4% as activated B-cell (ABC) subtype.According to Tally algorithm, 34. 4% were of GCB subtype and 65.6% of non-GCB subtype. Most of the patients (67. 8% ,61/90) received chemotherapy and 68.9% (62/90) underwent surgical resection. The overall 2, 3 and 5-year survival rates were 58. 5%, 52. 8% and 49. 8%, respectively. The overall 2, 3 and 5-year survival rates in the CHOP therapy group were 68.5%, 61.2% and 52. 9%, respectively.Conclusions There is no significant difference in ratio between the GCB and non-GCB/ABC subtypes by Hans and Choi algorithms. The non-GCB subtype seems to be more prevalent according to Tally algorithm.Although there is no significant difference in survival between GCB and non-GCB/ABC subtypes by the 3algorithms, GCB subtype tends to show a better survival. In univariate analysis, LDH level, international prognostic index, chemotherapy, surgical resection, B symptoms, number of involved sites and clinical stage are found to have prognostic significance. In multivariate analysis, Choi algorithm, Tally algorithm,chemotherapy, surgical resection, LDH level and clinical stage are independent prognostic factors.     

BCL-2、BCL-6、C-MYC基因重排的高级别B细胞淋巴瘤临床病理分析

目的探讨C-MYC、BCL-2和BCL-6基因重排的高级别B细胞淋巴瘤(high-grade B-cell lymphoma,HGBL)的病理形态、免疫表型及治疗方案。方法收集58例B细胞淋巴瘤患者临床资料,对其进行免疫组化及基因检测。结果 58例B细胞淋巴瘤中伴有C-MYC、BCL-2和BCL-6重排的HGBL仅1例,C-MYC和BCL-2、C-MYC和BCL-6重排各1例。3例患者均为中老年人,临床表现多为多部位受累,1例乳酸脱氢酶水平升高,Ann Arbor分期均为Ⅲ+Ⅳ期;生存期短。3例HGBL免疫组化检测均为生发中心B细胞型,Ki-67增殖指数均较高(90%～95%)。结论伴有BCL-2、BCL-6、C-MYC基因重排的B细胞淋巴瘤侵袭性强,常伴多部位及淋巴结外侵犯,分期晚,治疗反应差,诊断时需结合组织病理学和FISH基因检测结果,且需与其他类型的淋巴瘤相鉴别。     

脾脏非霍奇金淋巴瘤的临床病理特征及其与免疫表型的关系

目的 探讨脾脏非霍奇金淋巴瘤（NHL）的临床病理特征及其与瘤细胞属性的关系。方法 复习19例NHL的临床病理资料、进行随访、并用SP法行CD45RO、CD20及髓过氧化物酶等免疫组织化学染色,对CD45RO阳性的病例加作CD8、CD56、TLA-1、CD68免疫表型检测和EBER原位杂交。结果 （1）19例均有脾脏肿大,其中52.6%(10/19)有脾脏占位病变,（2）73.7%(14/19)为B细胞性,滤泡型5例,经济危弥漫型9例;中心母细胞性8例,中心母细胞/中心细胞性3例,小细胞性4例,10例原发脾脏NHL均为B细胞性;（3）26.3%(5/19)为外周T细胞性,大细胞性4例,小细胞性1例;TLA-1阳性3例,其中CD8阳性和CD56阳性各1例,且均为EBNER1/2阳性,余1例为CD8、CD56、EBER均阴性;均为继发脾脏NHL;（4）73.7%(14/19)有随访,9例生存者中有8例为原发脾脏NHL,生存时间为8个月-10年不等;5例死亡病例均为继发脾脏NHL,生存时间为2-6个月不等。结论 脾脏NHL的临床病理表现与瘤细胞的属性有一定关系。原发脾脏NHL的预后明显优于继发脾脏NHL,对原发脾脏NHL的诊断应从严把握。     

白血病和淋巴瘤免疫分型的研究

应用单克隆抗体ABC法、PAP法、酶组化等多标记方法研究白血病和淋巴瘤。其中急性白血病3例、恶性淋巴瘤4例、骨髓瘤3例、反应性增生2例、转移癌1例。本组发现1例急性单核——淋巴细胞混合型白血病,1例急性Tr淋巴细胞白血病/淋巴瘤。本文还讨论了MCAb和多标记研究在白血病、淋巴瘤的免疫分型、鉴别诊断的意义。     

间变性淋巴瘤激酶阴性的间变性大细胞淋巴瘤中C-MYC和PD-L1基因及蛋白表达的研究

目的:探讨C-MYC和PD-L1基因和蛋白在间变性淋巴瘤激酶阴性的间变性大细胞淋巴瘤（ALK －ALCL）患者中的表达,探讨两者在ALK －ALCL发病机制中的作用以及与临床病理特征的关系。 方法:收集福建省立医院病理科2003年1月至2017年1月ALK －ALCL患者3...     

Histiocyte-rich, T-cell-rich B-cell lymphoma: a distinct diffuse large B-cell lymphoma subtype showing characteristic morphologic and immunophenotypic features

AIMS: The clinicopathological features of histiocyte-rich, T-cell-rich B-cell lymphoma (HRTR-BCL) were first recognized in 1992. In this study, 60 cases of HRTR-BCL were analysed in order to provide a detailed morphological and immunophenotypical profile of the disorder. METHODS AND RESULTS: HRTR-BCL is easily distinguished from other B-cell lymphomas rich in stromal T-cells by (i) a diffuse or vaguely nodular growth pattern, (ii) the presence of a minority population of CD15-, CD20+ large neoplastic B-cells, (iii) a prominent stromal component composed of both T-cells and non-epithelioid histiocytes, and (iv) the scarcity of small reactive B-cells. These criteria also enable a reliable distinction from lymphocyte-rich classical Hodgkin's lymphoma (CHL), from lymphocyte-predominant Hodgkin's lymphoma (LPHL), paragranuloma type and from peripheral T-cell lymphoma. Based on the morphology of the neoplastic cells and on their frequent bcl-6 immunoreactivity, we speculate that HRTR-BCL may be derived from a progenitor cell of germinal centre origin. CONCLUSIONS: HRTR-BCL presents characteristic clinical features, affecting predominantly middle-aged men who present with advanced stage disease and are at high risk of treatment failure. Considering these distinctive clinicopathological features, recognizing HRTR-BCL as a lymphoma entity may be justified.     

结内外弥漫性大B细胞淋巴瘤的免疫表型和分化特征及预后的比较

目的探讨弥漫性大B细胞淋巴瘤（DLBCL）的临床生物学特征和预后并比较结内与结外的差异。方法分析142例DLBCL的临床病理资料,结内90例,结外52例（胃肠30例,其他22例）,并随访2～108个月,制备组织芯片,并经免疫组织化学EnVision法染色,观察CD10、bcl-6、MUM1蛋白的表达并进一步区分其生发中心B细胞（GCB细胞）和非生发中心B细胞的分化特征。结果胃肠道DLBCL常为Ⅰ～Ⅱ期,国际预后指标评分低,预后也好于结内及其他结外DLBCL。单个抗原的表达率,CD10为19％（27例）,bcl-6为51％（72例）,MUM1为58％（82例）。36％（51例）的DLBCL显示GCB细胞分化特征,64％（91例）的DLBCL显示非GCB细胞分化特征。结外DLBCL的bcl-6的表达（63％）高于结内DLBCL（43％）。在不同的结外部位,甲状腺等部位多见为GCB细胞分化的DLBCL;睾丸等部位多见为非GCB细胞分化的DLBCL。结论DLBCL显示生发中心B细胞和非生发中心B细胞分化特征,结内外以及结外不同部位的DLBCL有着不同的生物学特征和预后。     

Comparative clinicopathological study of primary CNS diffuse large B-cell lymphoma and intravascular large B-cell lymphoma

Primary CNS diffuse large B-cell lymphoma (CNS DLBCL) is confined to the CNS, and constitutes a distinct entity. In the present study a series of 40 Japanese patients with CNS DLBCL who presented with neurological, but not systemic symptoms, was reviewed. Median survival was 18.7 months. CD5, CD10, Bcl-6, MUM-1, and Bcl-2 were positive in 30%, 10%, 84%, 100%, and 93% of patients, respectively. All CD10-negative patients had non-germinal center B-cell type. There was no significant difference in survival among the immunophenotypic subgroups. CNS DLBCL appeared to be homogenous as a group, which prompted the comparison with another distinct extranodal entity, intravascular large B-cell lymphoma (IVLBCL) in Japanese patients. CNS DLBCL patients did not differ in age, sex, or immunophenotype, including CD5 positivity, from IVLBCL patients, but were significantly less likely to have poor prognostic parameters than IVLBCL patients: the international prognostic index score was low or low–intermediate in 86% of CNS DLBCL patients and high or high–intermediate in 98% of IVLBCL patients. Notably, despite this difference, their survival curves almost overlapped. The present study highlights the issue of clinical distinctiveness of aggressive extranodal lymphomas, the peculiar migration and localization of which should be further clarified.     

ALK阳性大B细胞淋巴瘤3例临床病理及分子遗传学分析

目的探讨间变性淋巴瘤激酶(anaplastic lymphoma kinase,ALK)阳性的弥漫性大B细胞淋巴瘤(diffuse large B-cell lym-phoma,DLBCL)的临床病理及分子遗传学特点。方法对3例ALK(+)DLBCL进行光镜观察、免疫组化及荧光原位杂交检测,并复习相关文献。结果镜下观察淋巴结结构破坏,可见淋巴窦侵犯,肿瘤细胞大,呈免疫母细胞样,免疫组化示肿瘤细胞ALK、Bob-1、CD4、CD10、CD45、CD56、CD138、EMA、MUM1、Oct-2均(+),CD20、CD79a、PAX-5、CD3和CD30均(-),3例荧光原位杂交均检测到ALK基因易位。结论 ALK(+)DLBCL是一种DLBCL的少见独立亚型,具有特征性免疫表型、形态学及分子遗传学特点。     

弥漫性大B细胞淋巴瘤基因表达谱的初步研究

目的探讨弥漫性大B细胞淋巴瘤（DLBCL）不同免疫表型组的基因表达谱状况。方法根据CD10、bcl-6和MUM1的表达状况对156例DLBCL进行分组：CD10^＋和（或）bcl-6^＋、MUM1-（第1组）;CD10^＋和（或）bcl-6^＋、MUM1^＋（第2组）;CD10^-和bcl-6^-、MUM1^＋（第3组）。从各组中各选择3例共（9例）临床分期为Ⅳ期的病例标本,另取3例正常扁桃体组织作为对照,采用Affymetrix U133 plus2．0寡核苷酸芯片研究12例样本的基因表达谱。结果通过unsupervised等级聚类分析,12例样本被分成了4组,分别命名为A、B、C、D组。经与免疫表型分组结果对照显示两种分组结果完全一致：A、B、C组分别对应第1、2、3组,D组对应正常对照组。在DLBCL病例组中（A、B、C组）有81个基因显著表达下调,有86个基因显著表达上调。而其中的一个组（B组）虽然具有混合性生发中心B细胞样（GCB,A组）和活化的外周血B细胞样（ABC,C组）DLBCL的免疫表型,但在聚类分析中发现其基因表达谱与A组和C组均不同,有45个基因表达上调,并且有27个特异性表达基因。结论初步结果显示,DLBCL全基因组表达谱在分子水平上有不同的亚群,且可能通过免疫表型来区分。还提示基因表达谱B组DLBCL可能存在除细胞起源以外的不同异质性因素,而这种因素可能与DLBCL的发病机制相关。     

Composite T lymphoblastic leukemia/lymphoma and diffuse large B-cell lymphoma: case report

This report concerns a unique case of a composite lymphoma composed of T-lymphoblastic leukemia/lymphoma (T-LBL) and diffuse large B-cell lymphoma (DLBCL) in a 72-year-old woman with generalized lymphadenopathy, splenomegaly and ascites. Laboratory findings showed increased lactate dehydrogenase and soluble interleukin-2 receptor. The biopsy specimen showed replacement of the normal architecture of the lymph nodes by a tumor containing a dual cell population composed of large lymphocytes and medium-sized lymphocytes. Sheets of large lymphocytes often were punctuated by clusters of medium-sized lymphocytes. Flow cytometry and immunohistochemical analysis showed a composite lymphoma with both T-LBL and DLBCL. The T-LBL expressed CD1a, CD3, CD4, CD8, and terminal deoxynucleotidyl transferase. The DLBCL expressed CD19 and CD20, CD23, bcl-2, bcl-6, MUM1 and immunoglobulin κ light chain. Polymerase chain reaction detected a monoclonal pattern of T-cell receptor γ and immunoglobulin heavy chain rearrangements in the same specimen. She received eight cycles of R-CHOP (rituximab+cyclophosphamide, doxorubicin, vincristine, prednisone) therapy and achieved complete remission. She has shown no signs of recurrence 20 months after the diagnosis. We describe here a very unusual and, to the best of our knowledge, an as yet never reported case of a primary composite lymphoma of T-LBL and DLBCL.     

间变性淋巴瘤激酶阳性的大B细胞淋巴瘤病理形态学及分子遗传学研究

目的 探讨间变性淋巴瘤激酶(ALK)阳性的大B细胞淋巴瘤的临床病理及分子遗传学特点.方法 对3例ALK阳性的大B细胞淋巴瘤进行光镜观察,采用免疫组织化学EliVision法及分子遗传学方法检测,并结合文献进行分析和讨论.结果 3例患者均为成年男性,年龄32～42岁,平均年龄36.3岁,病变累及淋巴结.镜下观察:淋巴结结构破坏,可见淋巴窦侵犯,肿瘤细胞大,呈免疫母细胞样/浆母细胞样形态.免疫组织化学示肿瘤细胞CD45、CD138、上皮细胞膜抗原、ALK阳性,且ALK蛋白染色呈胞质内颗粒状阳性,CD3、CD20、CD79a和CD30均阴性.3例间期荧光原位杂交均检测到ALK基因易位.结论 ALK阳性大B细胞淋巴瘤是弥漫性大B细胞淋巴瘤的少见独立亚型,好发于中年男性,病变部位以淋巴结多见,具有特征性形态学、免疫表型和分子遗传学特点.

Abstract：

Objective To study clinicopathologic and genetic features of anaplastic lymphoma kinase (ALK)-positive large B-cell lymphoma (LBCL). Methods Light microscopy, EliVision immunohistocheimical method and fluorescence in-situ hybridization were used to evaluate three ALK + LBCL cases recently diagnosed accompanied with a literature review. Results All three cases were male adult patients ( mean age = 36.3 years) with nodal involvement by lymphoma. Histologic evaluation revealed a diffuse effacement of the nodal architecture by the infiltration of tumor cells. Sinusoidal infiltration was seen.The neoplastic cells were large and exhibited the immunoblastic/plasmablastic morphology. By immunohistochemistry, all the cases showed a cytoplasmic granular staining of ALK. They were positive for CD45, CD138, and epithelial membrane antigen ( EMA), but were negative for CD3, CD20, CD79a and CD30. Fluorescence in situ hybridization (FISH) demonstrated the presence of ALK gene translocation in all of the cases. Conclusions ALK + LBCL represents a distinct variant of diffuse large B-cell lymphoma,usually involving lymph node of middle-aged men. The tumor has a immunoblastic/plasmablastic morphology along with a distinct immunophenotypic profile and ALK gene rearrangement.