眼附属器黏膜相关淋巴组织结外边缘区淋巴瘤A20及TNF基因的遗传学改变

 【摘要】 目的 检测眼附属器黏膜相关淋巴组织结外边缘区（MALT）淋巴瘤中A20基因及TNF基因数目的变化,为眼附属器MALT淋巴瘤发病机制的研究提供线索。方法 收集原发性眼附属器MALT淋巴瘤石蜡包埋组织标本41例, 利用间期荧光原位杂交（FISH）技术,使用商品化6号染色体着丝粒探针及自制位点特异性探针A20和TNF,检测眼附属器MALT淋巴瘤病例中6号染色体、A20以及TNF基因数目的异常。结果 41例样本中,A20杂合性缺失2例（4.88 %）;TNF基因多拷贝5例（12.20 %）,其中3例与6号染色体多体同时存在;无A20缺失与TNF多拷贝同时存在的病例。结论 中国少部分眼附属器MALT淋巴瘤中存在A20基因杂合性缺失及TNF位点多拷贝的遗传学改变, A20的缺失与TNF位点多拷贝无明显相关性。     

胃原发性MALT淋巴瘤临床病理分析

目的:探讨我区胃原发性黏膜相关淋巴组织型结外边缘区B细胞淋巴瘤(下称MALT淋巴瘤)的临床病理特点.方法:对14例胃原发性MALT淋巴瘤临床病理资料进行回顾性分析,并用免疫组化S-P法作下列抗体染色:HP、LCA、CD3、CD5、CD10、CD20、cyclinD1、1gM、κ、λ及BCL-2等.结果:男性5例,女性9例,中位年龄56岁.7例位于胃窦部,7例位于胃体.临床分期为:Ⅰ期8例(57%),Ⅱ期6例.l4例均表现为溃疡型病灶,病灶周围黏膜呈息肉样增生.组织学特点为:肿瘤细胞以边缘区B细胞为主,混有少量母化的细胞及浆细胞,可见淋巴上皮病变及反应性淋巴滤泡,7例(50%)可见向弥漫性大B细胞淋巴瘤转化区.免疫组化染色:HP阳性7例(50%),肿瘤细胞表达LCA、CD20、IgM;表达单一的轻链κ阳性5例、λ阳性9例;CD3、CD5、CD10、cyclinD1均阴性,中小肿瘤细胞BCL-2阳性,大细胞BCL-2阴性.结论:胃MALT淋巴瘤好发于中老年女性,易向弥漫性大B细胞淋巴瘤转化.     

胃原发性MALT淋巴瘤临床病理分析

目的：探讨我区胃原发性黏膜相关淋巴组织型结外边缘区B细胞淋巴瘤(下称MALT淋巴瘤)的临床病理特点。方法：对14例胃原发性MALT淋巴瘤临床病理资料进行回顾性分析，并用免疫组化S-P法作下列抗体染色：HP、LCA、CD3、CD5、CDIO、CD20、cyclinD1、lgM、κ、λ及BCL-2等。结果：男性5例，女性9例，中位年龄56岁。7例位于胃窦部，7例位于胃体。临床分期为：Ⅰ期8例(57％)．Ⅱ期6例。14例均表现为溃疡型病灶，病灶周围黏膜呈息肉样增生。组织学特点为：肿瘤细胞以边缘区B细胞为主．混有少量母化的细胞及浆细胞，可见淋巴上皮病变及反应性淋巴滤泡，7例(50％)可见向弥漫性大B细胞淋巴瘤转化区。免疫组化染色：HP阳性7例(50％)，肿瘤细胞表达LCA、CD20、IgM；表达单一的轻链κ阳性5例、λ阳性9例；CD3、CD5、CD10、cyclinD1均阴性，中小肿瘤细胞BCL-2阳性，大细胞BCL-2阴性。结论：胃MALT淋巴瘤好发于中老年女性．易向弥漫性大B细胞淋巴瘤转化。     

肺MALT淋巴瘤并腮腺淋巴上皮病1例及文献复习

目的：探讨肺黏膜相关淋巴组织型边缘区B细胞淋巴瘤（MALT-MZL）的临床表现、影像和病理学特点、治疗方法及其与淋巴上皮病（LEL）的关系.方法：报道1例肺MALT淋巴瘤并腮腺淋巴上皮病,对其进行病理组织学观察和免疫组织化检测,并回顾文献.结果：肺MALT淋巴瘤无明显临床表现,影像学特点为边界不清斑片影、结节影、团块影和叶段实变影,可见支气管充气征,缺乏特异性.病理示小淋巴细胞弥漫浸润,支气管、细支气管和肺泡上皮的浸润（淋巴上皮病变）是特征性的,但不特异.免疫组化可显示肿瘤的单克隆性,CD20和CD79a阳性.腮腺LEL是MALT淋巴瘤前期病变,能恶变为MALT淋巴瘤.结论：肺MALT淋巴瘤是一种罕见肿瘤,临床无特异性,确诊靠病理组织学和免疫组化检查,治疗以手术和化疗为主.LEL能恶变为MALT淋巴瘤.     

胃肠道外MALT淋巴瘤

目的:探讨胃肠道外MALT淋巴瘤临床病理特点.方法:复习14例胃肠道外MALT淋巴瘤临床病理资料及病理切片.结果:胃肠道外MALT淋巴瘤占MALT淋巴瘤的42.42%,发病中位年龄为50岁,85.71%为Ann Arbor Ⅰ期,42.86%发生于眼部.肿瘤细胞以边缘区B细胞样细胞为主,伴有少量免疫母细胞样细胞及浆细胞,1例可见大细胞转化区.随访11例,10例存活,1例死亡.结论:胃肠道外MALT淋巴瘤患者发病年龄较胃肠道患者年龄小,好发于眼部,较少向大细胞淋巴瘤转化.     

中国北方地区原发性眼附属器黏膜相关淋巴组织淋巴瘤染色体易位的间期FISH分析

目的 探讨淋巴瘤常见染色体易位在中国北方地区眼附属器黏膜相关淋巴组织(MALT)淋巴瘤中的发生率、意义及间期荧光原位杂交(FISH)方法在我国石蜡包埋组织中检测分子遗传学异常的可行性.方法 收集1995年4月至2007年8月原发性眼附属器MALT淋巴瘤石蜡包埋组织标本28份,米用IgH、MALT1(18q21)、bcl-6、c-myc、bcl-2、CCND1、bcl-10和FOXP1双色分离重排探针,利用问期FISH的方法,检测淋巴瘤常见染色体易位在眼附属器MALT淋巴瘤中的发生率.结果 所有标本均杂交成功.28份眼附属器MALT淋巴瘤标本中,IgH基因位点断裂1份,但其MALT1、bcl-6、c-myc、bel-2、CCND1、bcl-10和FOXP1基因均未见断裂,即未找到与IgH发生相互易位的伙伴基因.其余27份标本IgH、MALT1和bcl-6基因位点均未断裂,表明与MALT淋巴瘤相关的t(11;18)(q21;q21)/API2-MALT1、t(14;18)(q32;q21)/IgH-MALT1、t(1;14)(p22;q32)/bcl-10-IgH、t (3;14)(p14.1;q32)/FOXP1-IgH以及涉及bcl-6基因的染色体易位在本组病例中发生率为0.结论中国北方地区眼附属器MALT淋巴瘤中,罕见MALT淋巴瘤特异性染色体易位及其他淋巴瘤常见染色体易位,但可能存在新的涉及IgH基因的染色体易位.间期FISH方法是检测石蜡包埋组织分子遗传学异常的可靠方法.     

浆母细胞淋巴瘤1例报告及文献回顾

目的:探讨浆母细胞淋巴瘤（plasmablastic lymphoma,PBL）的临床病理特征及预后。方法:对1例鼻腔浆母细胞淋巴瘤进行光镜、免疫组化及原位杂交检测,并回顾分析63例文献报告病例。结果:肿瘤组织由中等或大细胞弥漫增生组成,胞浆少,嗜碱性,部分细胞胞浆嗜酸性伴核偏位,核圆形或卵圆形,核仁较明显,核分裂象易见,可见凋亡及肿瘤性坏死;免疫组化:瘤细胞CD79a、CD138、Plasma cell弥漫强（+）,Ki-67（约90%+）,LCA、CD20（-）;原位杂交:EBER（-）。结论:PBL在诊断标准、鉴别诊断及治疗方面仍存在争议,尚需积累更多病例进一步探讨。     

27例眼附属器淋巴瘤的临床病理特征分析

目的 探讨国人眼附属器淋巴瘤的临床病理特征。方法 回顾性分析解放军八一医院全军肿瘤中心1990年1月至2013年12月收治的27例经病理组织学检查确诊为眼附属器淋巴瘤患者的临床及病理学特征。结果 27例患者均为非霍奇金淋巴瘤,原发于眼部者25例,其他部位淋巴瘤累及眼部者2例。病理分型：NK/T细胞淋巴瘤2例;B细胞淋巴瘤25例,其中包括黏膜相关淋巴组织（MALT）淋巴瘤13例,淋巴浆细胞性淋巴瘤（LPL）4例,套细胞淋巴瘤（MCL）3例,小淋巴细胞性淋巴瘤（SLL）2例,髓外浆细胞瘤（EMP）3例。13例既往诊断为小B细胞淋巴瘤,经免疫组织化学染色,确诊为MALT淋巴瘤8例、MCL 3例和SLL 2例。结论 眼附属器淋巴瘤的病理诊断分类多样,明确诊断可以为患者治疗方案的选择和判断预后提供更合理的建议。     

25例眼附属器原发恶性淋巴瘤的特点及疗效

目的研究眼附属器原发恶性淋巴瘤的病理及临床特点,分析疗效,探讨减少放疗并发症的方法。方法眼附属器原发恶性淋巴瘤25例中,男11例,女14例。所有病例均经病理证实,其中19例行活检术,6例行肿瘤切除术。单眼发病22例,双眼同时受侵3例。肿瘤原发于眼睑或结膜16例（19眼）,原发于眶内其他部位9例。Ann Arbor分期ⅠE期2l例,ⅡE期3例,ⅢE期1例。22例（25眼）接受了放疗,采用180 kV深部X线或^（60）Coγ线或混合线照射,放疗剂量为30．0～57．6 Gy。结果全组5、10年生存率分别为90%、82%;原发于眼睑或结膜、原发于眶内的10年生存率分别为100%、58%（P=0．032）。肿瘤全切和肿瘤残留的10生存率分别为83%和82%（P=0．907）,局部控制率分别为80%和95%（P=0．369）。放疗组局部控制率为92%,未接受放疗组局部控制率为33%（P=0．006）。全组中22例为黏膜相关淋巴组织（MALT）淋巴瘤,2例大B细胞淋巴瘤,1例为T细胞淋巴瘤。MALT淋巴瘤和非MALT淋巴瘤的10年生存率分别为90%和33%（P=0．009）。全组中8眼发生放射诱发的白内障,7眼出现眼干燥症。结论眼附属器淋巴瘤放疗可以取得良好的效果,原发于眼睑、结膜者预后好于原发于眶内者。眼附属器淋巴瘤以MALT为主,其预后明显好于非MALT淋巴瘤者。放疗并发症主要为白内障和干眼症,通过合理设计放疗计划,给予适当的放疗剂量可有效降低并发症。     

胃肠道外MALT淋巴瘤

目的：探讨胃肠道外MALT淋巴瘤临床病理特点。方法：复习14例胃肠道外MALT淋巴瘤临床病理资料及病理切片。结果：胃肠道外MALT淋巴瘤占MALT淋巴瘤的42．42％，发病中位年龄为50岁，85．71％为Ann Arhot Ⅰ期，42．86％发生于眼部。肿瘤细胞以边缘区B细胞样细胞为主，伴有少量免疫母细胞样细胞及浆细胞，1例可见大细胞转化区。随访11例，10例存活，1例死亡。结论：胃肠道外MALT淋巴瘤患者发病年龄较胃肠道患者年龄小，好发于眼部，较少向大细胞淋巴瘤转化。     

CD20阳性血管免疫母细胞性 T 细胞淋巴瘤1例及文献复习

目的:探讨CD20阳性血管免疫母细胞性T细胞淋巴瘤（angioimmunoblastic T cell lymphoma,AITL）的临床特征及预后。方法:回顾分析我院1例CD20阳性AITL患者的临床病理特征、治疗转归并复习相关文献。结果:患者男性,69岁,以水肿及腹腔积液为首发表现,CT提示全身淋巴结肿大。免疫表型:CD20阳性、CD3(+)、CD5(+)、Ki-67(+,85%),其它B细胞标记阴性,EBER原位杂交阳性,TCR基因重排及IGH单克隆性重排阳性,多种治疗方案均无效。结论:CD20阳性AITL患者的临床病理特征易与B细胞淋巴瘤混淆,病理形态学、免疫组织化学及TCR基因重排检测等可减少误诊。利妥昔单抗及其他靶向药物的应用可能提高治愈率,改善患者预后。     

肾脏黏膜相关淋巴瘤,是慢性肾孟肾炎的后果吗?

Objective:To report 2 cases of primary renal marginal zone B-cell lymphomas of mucosa-associated lymphoid tissue (MALT lymphoma), and observe the relations between this rare tumor of kidney and chronic pyelonephritis. Methods: 2renal MALT lymphomas were collected from referral consultation. Detailed clinical information were reviewed, morphological analysis based on the HE section, and immunohistochemistry were performed by CD20, CD79a, CDS, CD10, CD43, CD23,BCL10 and Cyclin D1 antibodies. Results: 2 female patients with age of 48 and 55, respectively, all had a history of chronic pyelonephritis. Under the B ultrasonic and CT scanning a bump in the kidney was found. Renal carcinoma suspected and hereby the whole nephrectomy performed. In the macroscopic, tumors were laid in the renal medulla, with dark red color and ill-defined boundary. In the microscopic, there were mixed lymphoid cells infiltrate which mainly consisted of small lymphocytes, centrocyte-like cells, lymphoplasmacytoid and plasma cells, reactive follicles and lymphoepithelial lesions also could be seen in the lesion, but follicles colonization was rare. In fact, except changes of lymphoma, basic renal disease also could be seen. Most glomeruli were atrophic, some glomeruli were hyperplastic and hypertrophic. Tubules were dilated or contacted, many dilated tubules contained pink-color glassy-appearing casts that suggest the appearance of thyroid tissue.As a result, those 2 cases showed juxtaposed changes of lymphoma and pyelonephritis. Immunohistochemistry showed that tumor cells were CD20 and CD79a positive, CD43 was weak positive, but CD5, CD10, CD23, BCL10 and Cyclin D1 were all negative. Conclusion: Primary renal MALT lymphoma was very rare disease. According to the clinical manifestation, it's hard to differentiate from renal cell carcinoma. But the morphological features were consistent with the classic MALT lymphomas in other sites. Immunophenotypic profiles were helpful for diagnosis. Based on the truth that many MALT lymphomas in other sites were connected with chronic inflammations, we suppose that the renal MALT lymphoma may originate from chronic pyelonephritis.     

Primary renal mucosa-associated lymphoid tissue ymphoma, the result of chronic pyelonephritis？

Objective： To report 2 cases of primary renal marginal zone B-cell lymphomas of mucosa-associated lymphoid tissue （MALT lymphoma）, and observe the relations between this rare tumor of kidney and chronic pyelonephritis. Methods： 2 renal MALT lymphomas were collected from referral consultation. Detailed clinical information were reviewed, morphological analysis based on the HE section, and immunohistochemistry were performed by CD20, CD79a, CD5, CD10, CD43, CD23, BCL10 and Cyclin D1 antibodies. Results： 2 female patients with age of 48 and 55, respectively, all had a history of chronic pyelonephritis. Under the B ultrasonic and CT scanning a bump in the kidney was found. Renal carcinoma suspected and hereby the whole nephrectomy performed. In the macroscopic, tumors were laid in the renal medulla, with dark red color and ill-defined boundary. In the microscopic, there were mixed lymphoid cells infiltrate which mainly consisted of small lympho- cytes, centrocyte-like cells, lymphoplasmacytoid and plasma cells, reactive follicles and lymphoepithelial lesions also could be seen in the lesion, but follicles colonization was rare. In fact, except changes of lymphoma, basic renal disease also could be seen. Most glomeruli were atrophic, some glomeruli were hyperplastic and hypertrophic. Tubules were dilated or contacted, many dilated tubules contained pink-color glassy-appearing casts that suggest the appearance of thyroid tissue. As a result, those 2 cases showed juxtaposed changes of lymphoma and pyelonephritis. Immunohistochemistry showed that tumor cells were CD20 and CD79a positive, CD43 was weak positive, but CD5, CD10, CD23, BCL10 and Cyclin D1 were all negative. Conclusion： Primary renal MALT lymphoma was very rare disease. According to the clinical manifestation, it＇s hard to differentiate from renal cell carcinoma. But the morphological features were consistent with the classic MALT lymphomas in other sites. Immunophenotypic profiles were helpful for diagnosis. Based on the truth that many MALT lymphomas in other sites were connected with chronic inflammations, we suppose that the renal MALT lymphoma may originate from chronic pyelonephritis.     

肾脏黏膜相关淋巴瘤2例报道并文献回顾

背景与目的：原发肾脏的黏膜相关组织淋巴瘤十分罕见,目前世界报道不足50例。本文介绍2例肾脏黏膜相关淋巴瘤的形态学特点和免疫表型特征,旨在使临床和病理对这种低度恶性的B细胞肿瘤的特点有所了解。方法：收集病史资料,形态学评价根据HE切片,用免疫组化法检测肿瘤细胞的表型,使用的抗体包括CD20、CD79、CD5、CD10、CD43、CD23、BCL10和Cyc linD1。结果：2例患者均为女性,年龄分别为48岁和55岁,临床上均有慢性肾盂肾炎病史。B超和CT检查发现肾脏肿块,行全肾切除。大体检查可见肿块位于肾髓质,呈境界不清的暗红色;镜检见肾盂至肾实质弥漫淋巴样细胞浸润,以小淋巴细胞、中心细胞样细胞、淋巴浆细胞和浆细胞浸润为主,可见肿瘤细胞浸润肾小管和肾球囊形成淋巴上皮病变和反应性淋巴滤泡,但没有显著滤泡殖入;免疫组化显示增生细胞以B淋巴细胞为主,散在分布反应性T细胞,肿瘤细胞CD20、CD79 a阳性,CD43弱阳性,CD5、CD10、BCL10、CD23和Cyc linD1均为阴性。结论：原发肾脏黏膜相关淋巴瘤临床极为罕见,临床表现和辅助检查与肾细胞癌不易鉴别,但组织学特点符合经典黏膜相关淋巴瘤的所有特征,免疫表型有助于病理诊断。     

Translocations involving the immunoglobulin heavy chain gene locus predict better survival in gastric diffuse large B-cell lymphoma.

PURPOSE: The pathogenesis and clinical heterogeneity of gastric diffuse large B-cell lymphoma (DLBCL) are poorly understood. We have comprehensively investigated the incidence and clinical significance of lymphoma-associated chromosomal translocations, particularly those involving the immunoglobulin heavy chain (IGH) gene locus, in a large series of gastric DLBCL. EXPERIMENTAL DESIGN: One hundred forty-one cases of primary gastric DLBCL [58 with mucosa-associated lymphoid tissue (MALT) lymphoma and 83 without MALT lymphoma] were enrolled. Translocations involving BCL6, c-MYC, FOXP1, MALT1, and IGH were investigated using interphase fluorescence in situ hybridization. In positive cases, additional fluorescence in situ hybridization was done with appropriate probes for potential partner genes. Cases were classified into germinal center B-cell-like (GCB) or non-GCB subgroups by immunophenotyping with CD10, BCL6, and MUM1. RESULTS: Translocations involving IGH were detected in 36 (32%) of 111 cases; their partner genes included BCL6 (n = 10), c-MYC (n = 5), and FOXP1 (n = 3) but remained unknown in the remaining 18 cases. t(14;18)/IGH-BCL2, t(14;18)/IGH-MALT1, and t(1;14)/BCL10-IGH were not detected in any case. t(11;18)/API2-MALT1 was detected in none of the cases, except for one case of DLBCL with MALT lymphoma, which showed positive signals only in MALT lymphoma cells. IGH-involved translocation was associated with younger age but not with any other clinicopathologic factors including GCB or non-GCB immunophenotypes. Cox multivariate analysis revealed that IGH-involved translocation, in addition to younger age and early stage, was an independent prognostic factor for better overall and EFSs. CONCLUSION: IGH-involved translocations are frequent in gastric DLBCL and seem to identify cases with favorable prognosis.     

Clinicopathologic Assessment of Ocular Adnexal Lymphoproliferative Lesions at a Tertiary Eye Hospital in Iran

Background The most common type of ocular lymphoma is nonHodgkin lymphoma (NHL) categorized into two groups indolent (slow growing) and aggressive (rapid growing) Differentiating benign reactive lymphoid hyperplasia (RLH) from malignant ocular adnexal lymphoma (OAL) is challenging Histopathology immunohistochemistry (IHC) and ow cytometry have been used as diagnostic tools in such cases Materials and Methods In this retrospective case series from 2002 to 2013 at Farabi Eye Center 110 patients with ocular lymphoproliferative disease were enrolled Prevalence anatomical locations mean age at diagnosis and the nal diagnosis of the disease with IHC were assessed. Comparison between previous pathologic diagnoses and results of IHC was made Immunoglobulin light chains and Bcell and Tcell markers and other immunophenotyping markers including CD20 CD3 CD5 CD23 CD10 CYCLIND1 and BCL2 were evaluated to determine the most accurate diagnosis The lymphomas were categorized based on revised EuropeanAmerican lymphoma (REAL) classi cation Results Mean ageSD (years) of the patients was 556 193 and 61 were male Patients with follicular lymphoma large Bcell lymphoma or chronic lymphocytic leukemiasmall cell lymphoma (CLLSLL) tended to be older Nine patients with previous diagnoses of low grade Bcell lymphoma were reevaluated by IHC and the new diagnoses were as follows extranodal marginal zone lymphoma(EMZL) (n1) SLL(n1) mantle cell lymphoma (MCL) (n3) reactive lymphoid hyperplasia RLH (n2) Two cases were excluded due to poor block Flowcytometry reports in these sevenpatients revealedSLL with positive CD5 and CD23 MCLwith positive CD5 and CyclinD1 and negative CD23 EMZL with negative CD5CD23 and CD10. One RLH patient wasnegative for KappaLambda and positive for CD3 and CD20 and the other was positive for all of the light chains CD3 and CD20 Orbit (491) conjunctiva (161) and lacrimal glands (161) were the most common sites of involvement Conclusions Accurate pathological classi cation of lesions is crucial to determine proper therapeutic approaches This can be achieved through precise histologic and IHC analyses by expert pathologists