原发性鼻腔淋巴瘤临床病理及CT征象分析

目的 分析原发性鼻部非霍奇金淋巴瘤的临床病理与CT特点.方法 应用免疫组化方法和体层摄影术对36例原发性鼻部非霍奇金淋巴瘤患者的临床病理及CT资料进行回顾性分析.结果 病理类型主要以NK/T细胞淋巴瘤(21例)为主,其次是T细胞淋巴瘤(非特异型)(8例)、弥漫性大B细胞淋巴瘤(4例)、浆细胞瘤(2例)和黏膜相关淋巴瘤(1例).患者早期症状不典型;CT图像表现为鼻腔软组织密度影,部分患者病灶周围出现骨质破坏或吸收,增强扫描呈轻中度均匀强化.结论 原发性鼻部非霍奇金淋巴瘤以NK/T细胞型为主,临床表现缺乏特异性;CT表现可以显示病变部位及周围关系,有助于病变分期,确诊仍然依靠病理.     

北京协和医院对18年间病理诊断肺淋巴瘤患者的回顾分析

目的回顾性总结分析肺淋巴瘤的分类、分型特征及临床特点。方法收集北京协和医院1999年-2016年近18年期间病理诊断为肺淋巴瘤的全部病例,通过光镜、免疫组织化学染色及基因重排方法进行病理分析。结果本组共有肺淋巴瘤病例142例,男女比例1︰1.33,平均年龄48岁。123例(86.6%)诊断为非霍奇金淋巴瘤,19例(13.4%)诊断为霍奇金淋巴瘤。101例(69.2%)诊断为B细胞性淋巴瘤,其中45.6%(46例)为黏膜相关淋巴组织淋巴瘤或边缘区淋巴瘤,18.8%(19例)为弥漫大B细胞性淋巴瘤,11.9%(12例)为淋巴瘤样肉芽肿病。22例诊断为T细胞性淋巴瘤,其中27.3%为外周T细胞淋巴瘤(6例),13.6%(3例)为NK/T细胞淋巴瘤,9.1%(2例)为间变大细胞淋巴瘤。121例患者临床资料完善,其中64例(52.9%)为原发性肺淋巴瘤,男女比例1︰1.37,平均年龄50岁;57例(47.1%)为继发性肺淋巴瘤,男女比例1︰1.19,平均年龄45岁。在原发性肺淋巴瘤中,96.9%为非霍奇金淋巴瘤,3.1%为霍奇金淋巴瘤;在继发性肺淋巴瘤中,78.9%为非霍奇金淋巴瘤,21.1%为霍奇金淋巴瘤。65例手术切除或胸腔镜下肺活检病例全部进行了病理分类及分型,77例穿刺活检病例中有22例(28.6%)进一步分型困难。结论肺淋巴瘤中最常见类型依次为黏膜相关淋巴组织淋巴瘤、霍奇金淋巴瘤、弥漫大B细胞性淋巴瘤和淋巴瘤样肉芽肿病,其中黏膜相关淋巴组织淋巴瘤和淋巴瘤样肉芽肿病是主要的原发性肺淋巴瘤类型,而弥漫大B细胞性淋巴瘤和霍奇金淋巴瘤是主要的继发性肺淋巴瘤类型。开胸手术或胸腔镜下肺活检的病例易于明确病理分型,而穿刺活检病例中有小部分病例进一步分型困难。     

以中剂量阿糖胞苷为主方案治疗非霍奇金淋巴瘤的临床分析

目的观察以中剂量阿糖胞苷为主方案治疗非霍奇金淋巴瘤的治疗效果。方法对23例非霍奇金淋巴瘤患者以中剂量阿糖胞苷为主的方案进行化疗。其中T淋巴母细胞性淋巴瘤5例,B淋巴母细胞性淋巴瘤5例,原发性中枢神经系统弥漫性大B细胞淋巴瘤3例,难治复发的弥漫性大B细胞淋巴瘤3例,伯基特淋巴瘤2例,NK／T细胞性淋巴瘤2例,血管免疫母细胞性T细胞淋巴瘤1例、ALK阴性的间变性大细胞淋巴瘤1例,复发的套细胞淋巴瘤1例。结果完全缓解17例,部分缓解5例,无效1例。主要不良反应为骨髓抑制,Ⅲ-Ⅳ度白细胞和血小板减少的发生率分别为100％和78．2％。结论以中剂量阿糖胞苷为主的方案治疗非霍奇金淋巴瘤疗效较好,其骨髓的毒性反应也非常明显。     

胃原发性恶性淋巴瘤临床病理、免疫组化及超微结构研究

目的　探讨胃原发性恶性淋巴瘤的临床病理特点。方法　对 3 2例胃原发性非霍奇金恶性淋巴瘤的临床病理、免疫组化及超微结构进行观察。结果　 3 2例恶性淋巴瘤原发于胃底 3例 ,胃体 7例 ,胃角 8例 ,胃窦14例。全部病例做免疫组化染色 ,证实B细胞性淋巴瘤 3 1例 (96 9% ) ,T细胞性淋巴瘤 1例 (3 1% )。另外 ,对11例非霍奇金恶性淋巴瘤和 6例胃未分化癌进行了对比电镜观察 ,发现二者的超微结构有明显的差异。结论　绝大多数胃原发性恶性淋巴瘤为B细胞来源 ;免疫组化和超微结构观察对本病的诊断和鉴别诊断具有十分重要的意义。     

消化道原发性淋巴瘤36例临床病理分析

目的 探讨原发性消化道淋巴瘤的临床病理特点、诊断及鉴别诊断。方法对36例病理诊断为消化道原发性淋巴瘤。的病例临床资料、免疫组化进行回顾性分析研究。结果发生在胃部20例（56％），肠道13例（36％），阑尾3例（8％）。溃疡型13例（36%），浸润型15例（42％），结节型8例（22％）。36例中黏膜相关淋巴瘤（MALT）26例（72％），弥漫性大B细胞淋巴瘤（DLBCL）8例（22％），肠型T细胞淋巴瘤2例（6％）。结论消化道淋巴瘤临床缺乏特异性，内镜活检易漏诊或误诊为癌。明确组织学分型有助于选择治疗方案，发生在消化道MALT淋巴瘤病变局限，不累及淋巴结或其它组织器官，术后辅以化疗或放疗预后较好、多数可以长期存活。     

原发性肠道T细胞淋巴瘤临床特点分析

背景:原发性肠道T细胞淋巴瘤是一类起源于肠上皮内T淋巴细胞的恶性肿瘤,过去被称为“肠道恶性组织细胞增生症”,近年来发现这一疾病的本质是肠道T细胞淋巴瘤。该病临床表现复杂,病程进展迅猛,内镜和消化道钡餐检查很难确诊。目的:了解原发性肠道T细胞淋巴瘤的临床特点。方法:对仁济医院1994年9月～2004年9月6例原发性肠道T细胞淋巴瘤的病史资料进行回顾性分析。结果:本组原发性肠道T细胞淋巴瘤患者年龄34～73岁,男女比例为5∶1。病变多位于空、回肠,可有结肠累及。临床表现以腹痛、腹泻、发热、消瘦为主,部分患者并发肠穿孔、肠梗阻、消化道出血,无患者伴有乳糜泻。内镜下3例患者表现为溃疡病灶,术中见溃疡5例,肿块1例。1例患者经术前内镜活检病理检查确诊,5例由术后病理检查确诊,4例曾被误诊为炎症性肠病。淋巴瘤细胞的免疫表型为白细胞共同抗原(LCA)( )、CD45RO( )、CD3( )、CD30(-)。所有患者均接受手术治疗,部分结合术后化疗。3例患者于术后3个月内死亡。结论:不伴有乳糜泻是本组原发性肠道T细胞淋巴瘤的特点之一。患者的临床表现以一些非特异性症状为主,常被误诊为炎症性肠病。内镜活检和手术标本的病理学检查是目前确诊原发性肠道T细胞淋巴瘤的主要依据。该病预后极差。     

原发性甲状腺淋巴瘤7例临床及病理分析

目的分析原发性甲状腺淋巴瘤的临床特点及预后因素。方法回顾性分析2004—2009年北京中日友好医院收治的7例原发性甲状腺淋巴瘤的临床病理资料。结果全部病例均经甲状腺手术后病理组织学确诊,6例为中老年女性。所有患者均有甲状腺结节,部分患者有压迫症状。4例病理类型为弥漫大B细胞非霍奇金淋巴瘤,3例病理类型为黏膜相关淋巴组织B细胞淋巴瘤。病理分型为黏膜相关淋巴组织B细胞淋巴瘤及年轻患者预后较好。结论中老年女性甲状腺结节的鉴别诊断中应特别注意原发性甲状腺淋巴瘤的可能性,年龄及病理分型是影响该病预后的因素。     

原发性肠道T细胞淋巴瘤误诊为炎症性肠病的原因分析

目的原发性肠道T细胞淋巴瘤和炎症性肠病临床表现和影像学表现相似，为了预防误诊误治，我们对被误诊为炎症性肠病的原发性肠道T细胞淋巴瘤的临床特征进行分析。方法收集我院十年来（1994—2004年）所有肠道T细胞淋巴瘤和曾被误诊为炎症性肠病的原发性肠道T细胞淋巴瘤的资料，共6例进行分析。结果肠道T细胞淋巴瘤临床上少见，原发性肠道T细胞淋巴瘤和炎症性肠病在临床表现影像学和内窥镜的大体表现颇为相似，内窥镜下的活检和病理学和免疫组化检查是目前确诊肠道T细胞淋巴瘤的主要依据。结论肠道T细胞淋巴瘤的发病率低使临床医生对此种疾病的认识不足。由于缺乏特异性检测手段，为了避免误诊炎症性肠病，提高肠道T细胞淋巴瘤的诊断，要注意以发热、腹泻、腹痛、腹胀等非特异性症状为主，治疗效果不佳的患者要考虑原发性肠道T细胞淋巴瘤的可能性。内窥镜下大多表现为多形性、多灶性、弥漫性和不规则性溃疡，要在有特征性表现的部位多取组织块，提高活检的阳性率。肠道T细胞淋巴瘤易并发消化道穿孔、肠梗阻、消化道出血，诊断不明确时，必要时尽早剖腹探查以明确诊断。     

老年胃恶性淋巴瘤的临床特点与病理学分型

目的 探讨老年人胃恶性淋巴瘤的临床特点与病理学分型.方法 回顾性分析25例老年人胃恶性淋巴瘤病历资料,对临床特点、胃镜结果及病理分型等进行统计分析.结果 本组病例中,大部分患者有上腹痛、消瘦、消化道出血等表现,内镜表现有溃疡型、结节型、弥漫浸润型、肿块型,病理类型主要为B细胞非霍奇金淋巴瘤.结论 老年人胃恶性淋巴瘤的临床特点以消化道症状为主,但无特异性,内镜所见易误诊、漏诊,病理组织学检查是诊断金标准.     

以结肠多发溃疡为表现的肠病型T细胞淋巴瘤1例报道

肠病型T细胞淋巴瘤(enteropathy associated T-cell lymphoma,EATL)是一种原发于肠道的可能来源于肠道上皮内T淋巴细胞的结外T细胞淋巴瘤,临床少见,在欧美和亚洲的报道中均仅占全部非霍奇金淋巴瘤(non-Hodgkin’s lymphoma,NHL)的不足1%。临床上该病诊断较为困难,内镜下表现与肠结核、克罗恩病、肠型白塞病、结肠癌等病变难以区分,临床上具有较高的误诊、误治率。近期收治1例EATL患者,通过对该病例诊治过程的分析讨论,以期提高医务工作者对该病的重视及认识,避免临床误诊、误治。     

Changing incidence of lymphoproliferative disorders in Yorkshire

Secular trends in the incidence of lymphoproliferative disorders on North and West Yorkshire and Humberside from 1985 to 94 were studied and changes in incidence by tumour subtype were analysed. Population-based data on the incidence of lymphoproliferative disorders were obtained from a specialist registry with a high level of ascertainment. Cases of chronic lymphocytic leukaemia and plasma cell myeloma were excluded and the remaining cases classified as Hodgkin's disease and non-Hodgkin's lymphoma (NHL). NHL were subdivided by site of origin and immunophenotype. Nodal B-cell lymphomas were further classified as diffuse large B-cell lymphoma, follicle centre lymphoma, mantle cell lymphoma and miscellaneous. During the study period there was a significant increase in total lymphoproliferative disorders with an average change of 2.5% per annum equivalent to 0.84/10 0000. Most of this increase was due to an increasing incidence of extranodal B-cell lymphomas and peripheral T-cell lymphomas. A numerically small but significant increase in diffuse large B-cell lymphomas was seen. There was no significant increase in other subtypes. The increased incidence of lymphomas in the area studied is mainly due to changes in two specific subgroups. There are several reasons why changes in extranodal B-cell lymphoma and peripheral T-cell lymphoma may have been particularly affected by changing diagnostic practices. Epidemiological studies of particular subtypes of lymphoproliferative disorder facilitate the identification of environmental factors involved in the pathogenesis of these tumours.     

肠道弥漫性大B细胞淋巴瘤的CT表现

目的探讨肠道弥漫性大B细胞淋巴瘤的CT表现特点,提高对该病的诊断准确性。 方法收集空军军医大学西京医院2011年1月至2016年12月经病理、手术或多学科会诊最终诊断为肠道弥漫性大B细胞淋巴瘤的16例患者的临床、肠镜、病理及CT等资料,回顾性分析、总结其CT表现特点并对比诊断准确性。 结果94%(15/16)为单发病变,仅1例为多发;病灶41%(7/17)位于回盲部。肿瘤41%(7/17)表现为肠壁肿块样增厚合并肠腔扩张,35%(6/17)表现为肿块样,肿块样增厚合并肠腔狭窄、肠壁轻度局限性增厚各占12%(2/17)。13例肿块内见"血管漂浮征",肿块密度均匀14例,内有坏死3例,16例肿块均为轻-中度强化,1例强化明显。14例见周围、腹膜后或肠系膜淋巴结肿大。5例见肠外侵犯。 结论肠道弥漫性大B细胞淋巴瘤的CT表现具有一定特点,总结并掌握这些特点可以提高诊断的准确性。     

免疫组化联合基因重排检测对发热待查伴骨髓分类不明细胞的诊断价值

目的:探讨发热待查伴有骨髓中发现分类不明细胞免疫组化联合基因重排检测的诊断价值。方法:对23例长期发热并骨髓或外周血中有分类不明细胞浸润的患者分离骨髓或外周血的单个核细胞进行免疫组化染色和基因重排检测。结果:23例患者中,诊断为非霍奇金淋巴瘤12例,其中滤泡性淋巴瘤(FL)3例、套细胞淋巴瘤(MCL)1例、弥漫性大B细胞淋巴瘤(DLBCL)2例、间变性大细胞淋巴瘤-T(ALCL)3例、血管免疫母细胞性T细胞淋巴瘤(AITCL)1例,侵袭性NK细胞白血病/淋巴瘤(ANKCL)1例,恶性组织细胞病1例;2例结合脾脏病理学诊断SLE;诊断为骨髓转移癌5例;4例未能确诊。结论:联合运用免疫组化和基因重排技术对长期发热并骨髓分类不明细胞浸润患者有一定的诊断价值。     

Cytogenetic and clinicopathological characterization by fluorescence in situ hybridization on paraffin-embedded tissue sections of twenty-six cases with malignant lymphoma of small intestine

OBJECTIVE: In small intestinal malignant lymphoma (SIML), the correlation between specific chromosomal abnormalities and clinicopathological features remains unclear. The aim of this study was to determine the frequency of chromosomal translocations involving the BCL1, BCL2, c-MYC, BCL6 and MALT1 genes by using fluorescence in situ hybridization directly on paraffin-embedded tissue sections (tissue-FISH). MATERIAL AND METHODS: Twenty-six cases diagnosed as having SIML between 1996 and 2003 were the subjects of the clinicopathological investigation conducted in this study. Tissue-FISH was performed with specific probes on paraffin-embedded tissue sections as described previously. RESULTS: The primary site was frequently located at the duodenum (9 cases, 35%). In accordance with the World Health Organization classification, 14 (53%) cases were diagnosed as having diffuse large B-cell lymphoma (DLBCL) and 6 (23%) as marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT). Macroscopically, DLBCL and MALT lymphoma displayed various macroscopic features. Cytogenetically, IGH-BCL2 translocation was detected in 3 (21%) out of 14 DLBCL cases, but in none of the MALT lymphomas. BCL6 translocation was detected in 5 (35%) of 14 DLBCL cases and in 1 (17%) of 6 MALT lymphoma cases (17%). API2-MALT1 translocation was detected in 1 (7%) of 14 DLBCL cases and in 1 (17%) of 6 MALT lymphoma cases. CONCLUSIONS: The duodenum was preferentially involved in SIML. DLBCL and MALT lymphoma showed various macroscopic features. Tissue-FISH analysis disclosed that DLBCL is cytogenetically heterogeneous. Furthermore, our study validated tissue-FISH as an additional promising diagnostic tool for detecting specific chromosomal translocations in NHL.     

Immunoglobulin light chain gene translocations in non-Hodgkin's lymphoma as assessed by fluorescence in situ hybridisation

In non-Hodgkin's lymphoma (NHL), the majority of translocations involve the immunoglobulin heavy chain gene ( IGH ) locus, while a few involve the immunoglobulin light chain gene ( IGL ) locus, consisting of the kappa light chain gene ( IGκ ) and the lambda light chain gene ( IGλ ). Although many reports have dealt with the translocation and/or amplification of IGH in NHL, only a few have identified IGL translocations. To identify cytogenetic abnormalities and the partner chromosomes of IGL translocations in NHL, we performed dual-colour fluorescence in situ hybridisation (DC-FISH) and spectral karyotyping (SKY) in seven NHL cell lines and 40 patients with NHL. We detected IGL translocations in two cell lines and nine patients: four patients with diffuse large B-cell lymphoma, three with follicular lymphoma, one with extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue and one with mantle cell lymphoma. Five distinct partners of IGλ translocation were identified by SKY analysis: 3q27 in three patients, and 1p13, 6p25, 17p11.2 and 17q21 in one patient each. Three cases featured double translocations of IGH and IGL. These findings warrant the identification of novel genes 1p13, 6p25, 17p11.2 and 17q21.     

Adolescent non-Hodgkin lymphoma and Hodgkin lymphoma: state of the science

Lymphoma is the most common malignancy among adolescents, accounting for >25% of newly diagnosed cancers in the 15–19 year age group. Hodgkin lymphoma (HL) accounts for the majority (two-thirds) of cases, while the remainder of patients have one of four subtypes of non-Hodgkin lymphoma (NHL): diffuse large B-cell lymphoma (DLBCL) including primary mediastinal B-cell lymphoma (PMBL), Burkitt lymphoma (BL), lymphoblastic lymphoma (LL) or anaplastic large cell lymphoma (ALCL). Epidemiology, histology, treatment and outcome differ between HL and NHL, as well as among the various subtypes of NHL. Adolescent lymphoma is particularly interesting because it often shares features with both childhood and adult lymphoma. As medical oncologists and paediatric oncologists often follow divergent treatment plans, disagreements may arise between practitioners as to how best treat the adolescent group. Additional complicating factors associated with the adolescent years, such as lack of insurance, issues pertaining to body image, and concerns about fertility, can also hinder prompt, appropriate medical management. This review details the complexities associated with the diagnosis and treatment of adolescent lymphoma and updates the state of the science, with particular emphasis on epidemiology, diagnosis, and proper management of HL and the various subtypes of NHL.     

Lymphoma genesis in the context of HIV infection

The incidence of lymphomas is high among HIV infected patients. These lymphomas are non-Hodgkin's lymphoma (NHL) in 70% of cases and Hodgkin's disease (HD) in 30% of cases. Their localization is often extra-nodal with early dissemination. B-cell high grade NHL predominates. The most frequent histological types are diffuse large B-cell lymphoma (30 to 40%) and Burkitt's lymphoma (40 to 50%). Other histological types are low-grade B-cell lymphoma, polymorphic B cell lymphoma and primary effusion lymphoma. Three main factors are predominant in HIV-related lymphomagenesis: cellular immunodeficiency, oncogene viruses (Epstein-Barr and HHV8) and molecular lesions. HIV-related cellular immunodeficiency leads to the increase of EBV infected B-cells and to the diminution of antitumor immunity. Clonal EBV genome is found in lymphoma cells in 30 to 70% of cases of HIV-related NHL. It expresses oncogenic proteins including LMP-1 which behaves like an activated CD40. It induces the expression of intra-cellular genes which stimulate cell growth and inhibit apoptosis. Cytogenetic and molecular lesions are not specific to HIV-related NHL or to histological subtypes. A better knowledge of these mechanisms should lead to the development of specific targeted treatments (antiviral, cytotoxic anti-EBV lymphocytes, cell cycle regulators).     

Malignant lymphomas in coeliac disease: evidence of increased risks for lymphoma types other than enteropathy-type T cell lymphoma

BACKGROUND: Numerous studies have reported on the association between coeliac disease and the otherwise uncommon enteropathy-type T cell lymphoma (ETTL). A systematic risk assessment of more prevalent lymphoma entities, such as B cell and non-intestinal lymphomas, in coeliac disease has not been performed. AIMS: In light of the increasing number of patients diagnosed with coeliac disease and the unknown aetiology of malignant lymphomas, we aimed to estimate the distribution and risk of lymphoma subtypes in coeliac disease. METHODS: We reviewed and reclassified 56 cases of incident malignant lymphomas occurring in a Swedish population based cohort of 11,650 patients hospitalised with coeliac disease. The observed numbers of lymphoma subtypes were compared with those expected in the Swedish population. RESULTS: The majority (n=32, 57%) of lymphomas in the cohort were not intestinal T cell lymphomas. Significantly increased risks were observed for B cell non-Hodgkin lymphoma (NHL) (standardised incidence ratio (SIR) 2.2 (95% confidence interval (CI) 1.2-3.6); 11 non-intestinal and five intestinal) and for lymphomas of non-intestinal origin (SIR 3.6 (95% CI 2.3-5.2), 11 B and 14 T cell). Furthermore, 44% of patients with B cell NHL had a history of other autoimmune/inflammatory diseases. The relative risks for T cell NHL (SIR 51 (95% CI 35-68); n=37) and for primary gastrointestinal lymphomas (SIR 24 (95% CI 16-34); five B and 25 T cell) were markedly increased, as anticipated. CONCLUSION: Most lymphomas complicating coeliac disease are indeed related to the disease and are not of the ETTL-type. There was a remarkable aggregation of autoimmune/inflammatory disorders, female sex, coeliac disease, and B cell lymphoma.