肺黏膜相关淋巴组织边缘区B细胞淋巴瘤及良性淋巴组织增生性疾病的临床病理分析

目的 研究肺原发性黏膜相关淋巴组织边缘区B细胞(MALT)淋巴瘤及良性淋巴组织增生性疾病的临床病理形态、免疫组织化学表型和B细胞重链基因重排,比较肺MALT淋巴瘤和良性淋巴组织增生性疾病的差异.方法 回顾性的分析原发性肺MALT淋巴瘤13例,7例肺良性淋巴组织增生性疾病资料.对标本行常规HE染色,EnVision免疫组织化学染色(抗体包括AE1/AE3、CD20、CD79α、CD3、CD5、CD10、CD21、bel-2、bcl-6、cyclinD-1)及免疫球蛋白重链IgH基因重排检测.结果 13例肺MALT淋巴瘤,细胞成分多样,分别由不同比例的小淋巴细胞样细胞、中心细胞样细胞、单核样B细胞组成,常伴有浆细胞分化.肿瘤细胞以弥漫性和滤泡边缘区排列为主,常见反应性淋巴滤泡和滤泡中心的植入.肿瘤细胞呈串珠状直接侵犯肺泡间隔和沿支气管血管束向周边及肺膜扩散.MALT淋巴瘤中,均未见坏死.9例可见肿瘤细胞侵犯血管壁,6例可见胸膜累及,2例肺门淋巴结侵犯.9例肺MALT淋巴瘤可见淋巴上皮样病变,免疫组织化学显示上皮细胞内的淋巴细胞CD20阳性,CD3阴性.7例肺良性淋巴组织增生性疾病,2例可见淋巴上皮样病变,免疫组织化学显示,其淋巴上皮样病变内的淋巴细胞,部分CD20阳性,部分CD3阳性.9例肺MALT淋巴瘤进行了免疫球蛋白重链IgH基因重排,8例阳性;7例良性淋巴组织增生性疾病均为阴性.结论 肺MALT淋巴瘤在细胞组成和排列上与其他部位结外MALT淋巴瘤相同,肿瘤细胞呈串珠状直接侵犯肺泡间隔和沿支气管血管束向周边及肺膜扩散.在肺内淋巴上皮样病变常见于MALT淋巴瘤,并有助于诊断,但并非其特异性病变,一些肺的反应性淋巴组织增生也可出现,用免疫组织化学有助于区别两种病变.免疫球蛋白重链IgH基因重排可以帮助鉴别肺MALT淋巴瘤和良性淋巴组织增生性疾病.     

黏膜相关淋巴组织淋巴瘤

在呼吸道、消化道、泌尿生殖道黏膜及黏膜下存在无结构、散在的淋巴细胞。有结构的黏膜相关淋巴组织主要存在于回肠末端及支气管黏膜下。其最具特征的结构为Peyer斑（Peyer’s patches,1667年首先由Peyer描述而得名）。有结构的黏膜相关淋巴组织与淋巴结的淋巴组织结构相似,但无包膜。单个Peyer斑呈卵圆形,由生发中心、帽区及宽阔的边缘带B细胞构成。其外围为相邻的副皮质区样的T细胞区。边缘区的B细胞可进入覆盖Peyer斑的圆顶区上皮内（这些上皮内的B细胞与小肠其他部位上皮内的T细胞有别）。此外,固有膜内的浆细胞也是黏膜相关淋巴组织的一个组成成分。概括起来,     

黏膜相关淋巴组织淋巴瘤诊断标准

黏膜相关淋巴组织淋巴瘤(mucosa associated lymphoid tissue lymphoma,MALT-ML)的命名及诊断标准已在1997年WHO淋巴组织肿瘤新分类中确定[1],发表的有关文献也日渐增多.由于病理学工作者可能对此认识不足,导致误诊、漏诊者屡见不鲜,给患者带来不良后果.为此作者综合相关文献结合自己的多年工作体会将MALT-ML的概念与诊断标准作一介绍,供同道参考.     

胸腺黏膜相关淋巴组织结外边缘区淋巴瘤9例临床病理分析

目的探讨胸腺黏膜相关淋巴组织结外边缘区(extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue, MALT)淋巴瘤的临床病理学特征、免疫表型、分子特征及鉴别诊断。方法回顾性分析9例胸腺MALT淋巴瘤的临床资料、病理学形态、免疫表型及分子遗传学特征,结合文献对其临床病理学特点进行探讨。结果 9例胸腺原发MALT淋巴瘤患者年龄35～72岁(平均50岁),男性2例,女性7例。5例患者体检发现纵隔占位,2例表现为胸痛、咳嗽及四肢面部浮肿等症状。4例患者既往伴有自身免疫性疾病。镜下肿瘤主要由小到中等的淋巴细胞样细胞构成,弥漫浸润生长,其内见大小不等的囊肿形成,囊腔内可见胆固醇结晶沉积,瘤细胞侵犯囊壁及胸腺小体而形成淋巴上皮病变。免疫表型:9例肿瘤细胞均表达B细胞标志物(CD20、CD79α、Pax-5),基因重排均显示单克隆性B细胞增生,FISH检测未见MALT1基因断裂。结论胸腺MALT淋巴瘤较少见,属于低度恶性肿瘤,多数伴有自身免疫性疾病。单一形态的瘤细胞结合免疫表型及基因重排可明确诊断,警惕漏诊或过诊。手术完整切除后密切随访即可。     

黏膜相关淋巴组织淋巴瘤

黏膜相关淋巴组织淋巴瘤 (ｍｕｃｏｓａａｓｓｏｃｉａｔｅｄｌｙｍｐｈｏｉｄｔｉｓｓｕｅｌｙｍｐｈｏｍａ,ＭＡＬＴ ＭＬ )起初是指起源在胃肠道ＭＡＬＴ的一种Ｂ细胞淋巴瘤。早期的观点认为胃肠道的淋巴瘤基本都是ＭＡＬＴ ＭＬ ,并且是低度恶性的淋巴瘤 ,后来认识到有高度恶性的ＭＡＬＴ ＭＬ。但目前认为全身各处均可发生 ,无论有无ＭＡＬＴ ,只要有获得性ＭＡＬＴ/Ｐｅｙｅｒ小结样结构即可发生 ,小结中心为生发中心 ,周围有套细胞和边缘区细胞 ,故在黏膜层既可发生ＭＡＬＴ ＭＬ ,也可发生黏膜滤泡性淋巴瘤和黏膜套细胞淋巴瘤 ,因此…     

眼结膜黏膜相关淋巴组织边缘带B细胞淋巴瘤临床病理分析

目的 探讨眼结膜黏膜相关淋巴组织边缘带B细胞淋巴瘤(marginal zone B cell lymphoma of mucosa-associated lymphoid tissue)(简称为MALT淋巴瘤)的临床病理特征、治疗及预后.方法 对15例眼结膜MALT淋巴瘤患者的临床病理资料进行回顾性分析及随访,复查和完善HE及免疫组化染色切片,4例进行Ig基因重排克隆性分析.结果 (1)15例患者中,男性5例,女性10例,中位年龄42岁,病史平均20个月.(2)病理形态:黏膜下大量密集淋巴样细胞弥漫浸润,并有模糊淋巴滤泡样结节.浸润细胞多为小～中等大小的淋巴样细胞及单核样B细胞.(3)免疫表型:浸润细胞CD20、CD79a、BCL-2均(+),CD3、CD5、CD10、Cyclin D1、TdT均(-).(4)Ig基因克隆性分析:4例均呈单克隆.(5)随访:随访时间2～35个月,截止随访日期,所有患者均生存,且病变无复发.结论 眼结膜MALT淋巴瘤好发于中年女性,结膜红肿突起为主要特征,镜下以小细胞样边缘带B细胞为主,具有典型MALT淋巴瘤的免疫表型和惰性临床经过,预后良好.     

黏膜相关淋巴组织淋巴瘤和凋亡相关基因

细胞凋亡受抑是黏膜相关淋巴组织(mucosa-associated lymphoid tissue, MALT)淋巴瘤发生的重要机制。MALT淋巴瘤凋亡相关基因的研究逐渐引起了重视。API2-MALT1融合基因和bcl-10过表达分别激活NF-κB,以NF-κB激活为核心,涉及到p53,Fas,API2,MALT1等凋亡相关基因,导致细胞凋亡受抑。     

多脏器恶性肿瘤术后肝黏膜相关淋巴组织淋巴瘤

目的 探讨肝黏膜相关淋巴组织淋巴瘤的临床病理特征。方法 对1例罕见多脏器恶性肿瘤术后肝黏膜相关淋巴瘤病例结合文献进行临床、病理和免疫组化分析。结果 患者于8年和3年前先后发生胃恶性间质瘤、阴囊阴茎皮肤湿疹样癌，有长期化疗史。肿瘤组织学以单核样B细胞为主，并有淋巴滤泡和淋巴上皮病变形成。免疫表型示瘤细胞CD45、CD79α、CD20阳性，CD5、CD10、ALK、TdT阴性，bcl—2、Ki—67少数肿瘤细胞阳性。结论 肝黏膜相关淋巴瘤可以发生于多脏器恶性肿瘤术后，其发病可能与长期使用免疫抑制剂有关，诊断本病时需与肝继发性淋巴瘤及肝的炎性假瘤鉴别。     

33例粘膜相关淋巴组织起源的恶性淋巴瘤临床病理观察

33例粘膜相关淋巴组织(MALT)起源的恶性淋巴瘤,具有MALT的特点,发展缓慢,局限生长,主要由滤泡中心细胞和B_2样细胞构成,可见浆化和毛细血管后微静脉壁淋巴细胞浸润。特征改变是“淋巴上皮病变”。当侵犯性生长时,可出现绣毯结构。病变早期,B_2样细胞为主时,局部治疗有效,病人预后较好。     

Diagnostic features of gastric extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue

Gastric extranodal marginal zone B-cell lymphomas of mucosa-associated lymphoid tissue may be difficult to distinguish from florid gastritis and other small B-cell lymphomas. The following review details a practical summary of the morphologic features, immunohistochemical markers, and molecular tests that currently provide for an accurate diagnosis in daily practice.     

唾腺粘膜相关型淋巴瘤与良性淋巴上皮病变的临床病理学

目的：探讨唾腺粘膜相关型淋巴瘤（MALT－ML）与良性淋巴上皮病变（LEL）的诊断、鉴别诊断及其发病机制。方法：手术切除标本常规石蜡切片和免疫组化ABC法标记。结果：17例唾腺MALT－ML16例为CCL细胞型，1例为淋巴浆细胞型，具有MATL－ML的共同特征，免疫组化示单克隆性，7例LEL示多克隆性。结论：Ⅰ级、Ⅱ级LEL为良性病变，Ⅲ级为交界性病变。Ⅳ级LEL即MALT－ML，因此唾腺MALT－ML与LEL密切相关。     

An unusual presentation of primary pulmonary extranodal marginal zone lymphoma of mucosal associated lymphoid tissue: An autopsy case report

A 40?year old female with no documented medical history presented to the Emergency Department with several days of lethargy and altered mental status. She was found to be anemic, thrombocytopenic, and hypotensive. The patient was found to be in severe metabolic acidosis, became bradycardic, and quickly deteriorated. Clinicians suspected thrombotic thrombocytopenic purpura, and the diagnosis was supported by ADAMTS13 testing. The clinicians attempted to place a Quinton catheter for emergent plasmapheresis, but the patient expired before definitive treatment could be initiated. Autopsy was obtained and revealed a right middle lobe consolidation grossly consistent with lymphoid tissue or tumor.     

Thymic epithelial cells expressed unusual follicular dendritic cell markers: thymic extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue

Described herein is a case of thymic extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue. Using immunohistochemical double staining it was found that most of the thymic lymphoid follicles in this case possessed cytokeratin-positive and follicular dendritic cell (FDC) marker-positive cells. Moreover, using immunoelectron microscopy it was confirmed that some of the double-positive cells were thymic epithelial cells. The candidate of cytokeratin subtype expressed on the double-positive cells was cytokeratin 1 (CK1), which was expressed only by the epithelium of Hassall's corpuscles in thymuses from age-matched patients with myasthenia gravis. The present case indicates a possibility that some thymic epithelial cells become FDC, although it was uncertain whether they were derived from the epithelia of Hassall's corpuscles or whether they were at the same differentiation stage as Hassall's corpuscles.     

Paediatric marginal zone lymphoma and hyperplasia

Marginal zone lymphomas (MZL) are low-grade B-cell lymphomas arising from post-germinal memory B-cells occurring in adults with a slight female predilection. They are sub-categorized into nodal (NMZL), extra-nodal/mucosa-associated lymphoid tissue (MALT) and splenic (SMZL). MALT lymphomas are the most common (70%) followed by SMZL (20%) and NMZL (10%). Histologic transformation into aggressive B-cell lymphoma can rarely occur. MZL is extremely uncommon in the paediatric population and unlike in adults, is predominantly nodal. Paediatric NMZL (pNMZL) is an indolent, low-grade lymphoma with unique clinical and morphologic features. In contrast paediatric MALT lymphoma and SMZL are extremely uncommon and resemble their adult counterparts. Paediatric marginal zone lymphomas must be differentiated from paediatric-type follicular lymphoma (PFL) and marginal zone hyperplasia (MZH) of lymph nodes and mucosa-associated lymphoid tissue. This review summarizes the pathogenesis, morphology, genetic features of paediatric MZL and marginal zone hyperplasia. Recognition of these entities is important to avoid unnecessary therapy.     

Collision of EBV‐associated gastric carcinoma and primary gastric extranodal marginal zone lymphoma of mucosa‐associated lymphoid tissue in the remnant stomach

Reported herein is a case of EBV‐associated gastric carcinoma with primary gastric extranodal marginal zone lymphoma of mucosa‐associated lymphoid tissue (MALT lymphoma). A 69‐year‐old Japanese man was found to have an ulcer lesion in his stomach on endoscopy, and a biopsy indicated malignancy. He underwent gastrectomy. Microscopically the tumor had features typical of lymphoepithelioma‐like carcinoma. The neoplastic epithelial cells proliferated in a trabecular fashion. On in situ hybridization for EBV‐encoded RNA, positive signals were observed in most neoplastic epithelial cells. Numerous lymphocytes surrounded the neoplastic epithelial cells. In the stroma, numerous lymphocytes with mild atypia were positive for CD20 and CD79a. In addition, monoclonal proliferation of B cells was confirmed on polymerase chain reaction for IgH. These findings supported MALT lymphoma. The coexistence of EBV‐associated gastric carcinoma and MALT lymphoma is extremely rare.     

Primary extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue type with malakoplakia in the urinary bladder: a case report

Primary malignant lymphoma of the urinary bladder is a rare disease constituting less than 1% of neoplasms of the urinary bladder. The most prevalent histological subtype is extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue type (MALT lymphoma). It is frequently associated with chronic cystitis and predominantly occurs in females. On the other hand, malakoplakia is thought to be a reactive granulomatous lesion occurring most prevalently in the genitourinary tracts. It is frequently found in females and often associated with bacterial infection in immunosuppressive status. Here we report a rare case of concurrent primary MALT lymphoma and malakoplakia in the urinary bladder in a 78-year-old Japanese female. Presumably, both lymphoma and malakoplakia are considered to be involved in the antecedent cystitis and might contribute to the development of the urinary bladder tumor of the patient, leading to the occlusion of the right ureter with subsequent hydronephrosis.