脾边缘区淋巴瘤的诊断

目的:探讨脾边缘区淋巴瘤的临床特征、诊断及鉴别诊断。方法:回顾性分析华中科技大学同济医学院附属同济医院2019年收治的3例表现为脾大、血细胞减少的CD5 - CD10 - B细胞非霍奇金淋巴瘤患者的临床诊断及鉴别诊断过程,并复习相关文献。 结果:3例均为老年患者,均出现不同程度的脾大和血细胞减少,均在骨髓或淋巴结中发现CD5 - CD10 -单克隆B淋巴细胞。综合患者的临床特征、外周血及骨髓形态、免疫表型和遗传学特征,2例患者诊断为脾边缘区淋巴瘤,1例患者诊断为弥漫大B细胞淋巴瘤。 结论:脾边缘区淋巴瘤的诊断需综合临床特征、外周血及骨髓形态、免疫表型和遗传学特征,与其他CD5 - CD10 -小B细胞淋巴瘤进行仔细鉴别。新一代基因突变高通量检测和表达谱分析有助于不典型疑难病例的精准诊断。     

黏膜相关淋巴样组织型结外边缘区B细胞淋巴瘤的分子细胞遗传学研究进展

黏膜相关淋巴样组织型结外边缘区B细胞淋巴瘤（extranodal marginal zone B—cell lymphoma of mucosa-associated lymphoid tissue，MZL—MALT）是结外最常见的淋巴瘤，近年研究表明慢性感染或自身免疫性疾病所致的直接或间接感染及分子遗传学异常是MZL—MALT发生、发展的基础。     

肺黏膜相关淋巴瘤的诊断与鉴别诊断

目的 探讨肺黏膜相关淋巴组织边缘区B细胞淋巴瘤（MALToma）的临床、影像、病理学特征和可靠的诊断依据,并与形态非常相似的3种肺良性淋巴组织增生性病变相鉴别.方法 分析3例肺MALToma患者的病史、影像学、病理组织学等检查,与淋巴细胞性间质性肺炎、淋巴细胞性炎性假瘤、滤泡性支气管炎鉴别.结果 肺MALToma临床表现和影像学缺乏特异性,与肺癌不易鉴别.病理示边缘区淋巴细胞增生、围绕生发中心并相互融合,侵入支气管上皮形成“淋巴上皮病变”,侵入滤泡生发中心形成“滤泡植入”.结论 肺MALToma是一种少见的低度恶性肿瘤,临床症状和影像学无特异性,确诊需依据病理组织学.与其他肺淋巴组织增生性病变的主要鉴别点在于边缘带的范围大小、有无融合、有无“滤泡植入”、有无“淋巴上皮病变”、滤泡之间的细胞类型等.     

眼睑黏膜相关性淋巴组织结外边缘区B细胞淋巴瘤二例并文献复习

目的 探讨眼睑黏膜相关性淋巴组织结外边缘区B细胞淋巴瘤（MALT淋巴瘤）的临床病理特征、诊断与鉴别诊断、治疗及预后。方法 回顾性分析哈尔滨市第一医院2例眼睑MALT淋巴瘤的临床资料、组织病理学形态和免疫组织化学结果,并复习文献。结果 2例患者以眼睑肿物为首发症状,均在局部麻醉下完成眼睑肿物切除术,病理检查示肿瘤细胞形态较一致,中等大小,胞质丰富,呈弥漫性或结节状排列,诊断为眼睑MALT淋巴瘤。免疫组织化学示2例患者肿瘤细胞均CD20（＋）,CD79a（＋）,CD3（-）,CD10（-）,bcl-6（-）,CD5（-）,cyclin D1（-）,CK（-）。结论 眼睑MALT淋巴瘤临床表现缺乏特异性,病因与感染有关,目前尚无一致的治疗方案,要避免过度治疗;病理学检查及免疫组织化学对该病的诊断和鉴别诊断具有重要意义。     

小肠黏膜相关淋巴组织结外边缘区B细胞淋巴瘤1例报告

1临床资料患者,男,56岁。因"反复腹泻2年余",于2010年1月28日入住上海瑞金医院。患者2008年1月起,无明显诱因下出现腹泻,每次发作3-5天,每天腹泻7次左右,为黄色稀水样便,无黏液及脓血便,无腹痛,恶心、呕吐,伴发热,体温39℃-40℃左右,在当地医院予以左氧氟沙星等补液抗炎     

淋巴组织反应性增生与恶性淋巴瘤组织学鉴别诊断

淋巴组织反应性增生(KH)与恶性淋巴瘤(NHL与HL)组织学鉴别诊断最核心的问题是抓住KH复杂多变的组织形态的免疫反应本质,以量变为主,病原消失可以复原。而恶性淋巴瘤因发生了质的变化,瘤细胞产生了相对无限制的克隆性增生,有破坏性和转移性。HL更要注意以诊断性R-S细胞为代表的系列R-S细胞的形态分布特殊性。     

原发性肺结外边缘区B细胞淋巴瘤九例临床病理分析

目的 探讨原发性肺结外边缘区B细胞淋巴瘤(primary pulmonary extranodal marginal zone B-cell lymphoma，即黏膜相关淋巴瘤，mucosa-associated lymphoid tissue lymphoma，MALT淋巴瘤)的临床病理特征和免疫组织化学(immunohistochemistry，IHC)染色，及其诊断和预后。方法 对9例原发性肺结外边缘区B细胞淋巴瘤进行临床病理、免疫组织化学(LCA、CD20、CD45RO、κ、λ、Ki-67、CD5、cyclinDl、CK、NSE)分析。结果 结合免疫组织化学染色及病理组织形态，按淋巴造血组织肿瘤2001年新WHO分类，9例均被确诊为原发性肺结外边缘区B细胞淋巴瘤(MALT淋巴瘤)。本组男4例，女5例，男女之比为1：1．25；中位年龄51．5岁。所有MALT淋巴瘤病例都呈LCA阳性、CD20阳性，Ki-67阳性率低；免疫球蛋白轻链限制性λ或κ阳性表达者分别为5例、1例，κ和λ均阳性表达者1例，2例无轻链限制性表达；CD45RO、CD5、cyclinDl、CK、NSE均阴性。结论 原发性肺结外边缘区B细胞淋巴瘤属惰性淋巴瘤，临床极易误诊，必须结合其病理组织形态和免疫组织化学染色进行诊断和鉴别诊断。     

BIOMED-2体系检测免疫球蛋白重链基因重排在黏膜相关淋巴组织结外边缘区B细胞淋巴瘤诊断中的应用

 【摘要】　目的　探讨BIOMED-2标准化体系检测免疫球蛋白重链（IGH）基因重排对黏膜相关淋巴组织结外边缘区B细胞淋巴瘤（MALT淋巴瘤）诊断的应用价值。方法　45例标本包括不同部位原发的MALT淋巴瘤36例、淋巴结外淋巴组织增生性病变3例、幽门螺杆菌（HP）感染相关性重度胃炎6例。用DNA提取试剂盒从石蜡包埋组织中提取所选病例的基因组DNA,通过BIOMED-2体系质控混合引物检测其质量,应用IGH VH-JH 3组引物组合进行IGH基因克隆性重排检测;比较分析该体系在MALT淋巴瘤诊断中的敏感性和特异性。结果　45例中31例（MALT淋巴瘤22例,淋巴组织增生性病变3例、重度胃炎6例）提取的基因组DNA可扩增出300 bp片段,其余14例DNA均严重降解。22例MALT淋巴瘤中,16例检测出IGH基因重排,灵敏度为72.7 %;6例严重胃炎病例未检测出IGH克隆性重排,其特异度是100 %;3例淋巴组织增生性病变中1例检测出IGH克隆性重排,2例未检测出重排。结论　在MALT淋巴瘤的诊断和淋巴组织增生性疾病的鉴别诊断中,BIOMED-2标准化体系检测IGH基因克隆性重排是一种快速可靠的方法,具有重要的临床应用价值。     

Retreatment with chimeric CD 20 monoclonal antibody in a patient with nodal marginal zone B-cell lymphoma

A patient with advanced and chemotherapy-refractory nodal marginal zone B-cell lymphoma was given a course of chimeric CD 20 monoclonal antibody Rituximab. Partial response was observed without any major toxicities. Retreatment with Rituximab for disease progression six months after the first course led to partial remission. Adjuvant radiotherapy was given for the residual disease and, currently, patient's disease remains stable eight months after the second course of Rituximab. This case demonstrates the therapeutic efficacy and feasibility of retreatment with Rituximab for relapsed or refractory low grade lymphoma.     

Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue arising in the lateral ventricle

Extranodal marginal zone B-cell lymphomas of mucosa-associated lymphoid tissue (MALT) lymphomas are a well-described type of low-grade B-cell non-Hodgkin lymphoma. They typically arise adjacent to mucosal surfaces in the gastrointestinal tract, lung and conjunctiva, and, less frequently, in the skin, salivary gland and thyroid gland. Unusual locations, such as the genitourinary tract, thymus and meninges, have also been reported. We recently encountered a case of an intracranial MALT lymphoma in a 53-year-old man who presented with persistent headaches and a seizure. The lesion developed as a mass within the lateral ventricle, appeared to be arising from the choroid plexus, and was not associated with meninges. Histologically, there was a vaguely nodular, dense lymphoid infiltrate with occasional benign follicles colonized by marginal zone lymphoma, suggesting derivation from a focus of prior inflammation. Translocations involving the MALT1 gene were not identified but karyotypic evaluation highlighted a complex cytogenetic profile with many chromosomal abnormalities. This rare case provides insight into the pathophysiology of MALT lymphomas.     

Transformed diffuse large B-cell lymphoma from marginal zone lymphoma in the anterior mediastinum: A case report and review of the literature

Marginal zone lymphoma (MZL) arising from the anterior mediastinum is rare. In the majority of reported cases, the tumor was incidentally discovered, reflecting its indolent clinical features. We present a 38-year-old woman who had no medical history, and presented with a bulky anterior mediastinal tumor complicated by life-threatening compression of the vasculature and bronchi. Biopsy specimens of the neoplasm suggested transformed diffuse large B-cell lymphoma (DLBCL) from MZL. To our best knowledge, this is the first case report of anterior mediastinum MZL associated with an aggressive clinical course and life-threatening complications likely due to transformation to DLBCL.     

伴有淀粉样物质沉积皮肤边缘区B细胞淋巴瘤临床病理特点(英文)

Background and Objective:Amyloid deposition is rare.If there was a great amount of amyloid depositions in the skin tissue,it would be considered to be amyloid deposition disease at first,and then primary cutaneous marginal zone B-cell lymphoma(PCMZL).This study was to analyze the diagnosis and differential diagnosis of two cases of PCMZL with amyloid deposition.Methods:Clinicopathologic characteristics and follow-up of two cases of PCMZL were analyzed.Immunohistochemical staining was performed by EnVision m...     

免疫分型标记物和CD43在非特指弥漫性大B细胞淋巴瘤中的表达及临床意义

目的 探讨免疫分型标记物和CD43在非特指弥漫性大B细胞淋巴瘤(DLBCL NOS)中的表达及其临床意义.方法 收集120例DLBCL NOS临床病理资料进行分析.采用免疫组化法检测CD10、bcl-6、MUM-1和CD43的表达,根据Hans分型将其分为GCB型和non-GCB型.结果 120例DLBCL,NOS中GCB型和non-GCB型分别为38例和82例,Hans分型与DLBCL NOS预后无关(P＞0.05).120例DLBCL NOS中CD43阳性33例(27.5％),CD43表达与性别、临床分期和免疫分型均无关(P＞0.05),与年龄(P=0.036)和生存状态(P=0.004)有关.结论 DLBCL NOS预后差与CD43阳性表达有关,与Hans分型无关.     

眼附属器黏膜相关淋巴组织结外边缘区B细胞淋巴瘤（MALT）临床病理分析

目的:探讨6例眼附属器黏膜相关淋巴组织结外边缘区B细胞淋巴瘤(MALT)患者的临床病理特点及预后。方法:收集2010年01月至2019年01月病理学检查确诊的6例眼附属器MALT患者的临床资料及治疗方案、形态学特点及免疫组化表达,FISH检测MALT1基因断裂,电话随访,分析总结其临床病理特点及预后。结果:临床表现为眼部不适,眼睑肿胀及视物模糊等。镜下可见肿瘤由形态多样的小B细胞组成,包括边缘带细胞（中心细胞样细胞）、单核样细胞、小淋巴细胞,也可见散在的免疫母细胞和中心母细胞样细胞,部分细胞有浆细胞样分化。5例肿瘤细胞CD20（+）、CD3（-）、BCL2（+）、Ki67约10%~25%,3例BCL10及AEG1阳性,1例伴浆细胞分化,免疫组化表现为CD20（-）、CD79α（+）、CD38（+）、CD138（+）、MUM1（+）、Kappa、Lambda呈限制性表达,2例FISH检测结果阳性。结论:眼附属器MALT淋巴瘤常CD20、BCL2、BCL10、AEG1阳性,FISH可作为辅助诊断。     

Prognostic value of the Follicular Lymphoma International Prognostic Index score in marginal zone lymphoma

BACKGROUND:

In marginal zone lymphoma (MZL), clinical and follow‐up data on large cohorts of patients are difficult to obtain. The objective of this single‐center, retrospective analysis of a large cohort of 144 patients with MZL was to elucidate the role of prognostic markers, treatments, and outcomes in this disease.

METHODS:

In total, 144 patients were identified who were diagnosed with MZL at the authors' institution between 2003 and 2010. Data on clinical parameters, treatments, response, and survival were analyzed. In addition, the validity of the International Prognostic Index (IPI) and Follicular Lymphoma International Prognostic Index (FLIPI) prognostic scores were tested in patients with MZL.

RESULTS:

Among 144 patients with MZL, 96 patients (67%) had extralymph node (extranodal) MZL, 32 patients (22%) had lymph node (nodal) MZL, and 16 patients (11%) had splenic MZL. The 5‐year progression‐free survival rate was 82% in the nodal MZL group, 88% in the extranodal MZL group, and 74% in the splenic MZL group and did not different between the 3 groups (P = .60). The 5‐year overall survival rate was excellent in all 3 MZL groups (nodal MZL, 89%; extranodal MZL, 92%; splenic MZL, 82%; P = .46). In our cohort, the FLIPI score was a significant prognostic marker: The 5‐year progression‐free survival rate for patients who had FLIPI scores of 0 to 2 (low or intermediate risk) was excellent at 92%, whereas it was only 62% for patients who had FLIPI scores of 3 to 5 (poor risk; P = .003). Similarly, the 5‐year overall survival rate for patients who had FLIPI scores of 0 to 2 was 95%, whereas it was only 62% for patients who had FLIPI scores of 3 to 5 (P = .0009).

CONCLUSIONS:

The FLIPI score had strong prognostic value in patients with MZL. Patients who have low‐risk or intermediate‐risk FLIPI scores have an excellent prognosis, whereas patients with poor‐risk FLIPI scores are candidates for novel treatment approaches. Cancer 2013. © 2012 American Cancer Society.