阑尾原发Burkitt淋巴瘤临床病理观察

目的：探讨阑尾原发Burkitt淋巴瘤（BL）的临床病理特点、免疫表型及分子生物学特征。方法：运用组织形态及免疫组化,EB病毒（EBER）原位杂交方法研究1例发生在阑尾的Burkitt淋巴瘤,并结合文献进行分析讨论。结果：阑尾Burkitt淋巴瘤（BL）形态学表现为肿瘤细胞弥漫一致性增生的中等大小细胞浸润,部分瘤细胞周围空晕伴有星空现象。免疫组化显示,肿瘤细胞LCA,CD20及CD10均为阳性,Ki-67大于80%阳性,CD99,CD3p,Mpo阴性,原位杂交EBER阴性。结论：原发于阑尾Burkitt淋巴瘤（BL）非常罕见,属高度侵袭性。应与其他类型淋巴瘤相鉴别,需结合组织形态及免疫组化,原位杂交等为临床诊断和及时治疗提供依据。     

散发性Burkitt淋巴瘤的病理学特点

 目的　探讨散发性Burkitt 淋巴瘤（BL）的临床病理、免疫表型及分子生物学特征。方法　对20例散发性BL病例进行了光镜、免疫组化、EB病毒（EBV）原位杂交及间期荧光原位杂交（FISH）检测,结合临床特征进行综合分析。结果　20例散发性BL中,男性16例,女性4例;年龄3～14岁,中位年龄9岁。光镜下部分典型的BL肿瘤细胞弥漫一致性增生浸润,见较明显的吞噬核碎片的巨噬细胞形成的"星天现象"。部分病例形态学需鉴别其他肿瘤。免疫组织化学染色显示,瘤细胞表达CD20和CD10,不同程度地表达CD79a、bcl-6、MUM-1等,大于95 %的瘤细胞Ki-67阳性;EBV原位杂交显示EBER 1／2 阴性;间期FISH有c-myc基因异常。结论　散发性BL属高度侵袭性淋巴瘤,需要与多种其他肿瘤鉴别,明确诊断有助于临床针对性地进行高强度治疗。基于分子水平及蛋白水平多项标志物的检测,可大大减少漏诊和误诊,并为临床的准确诊断和及时治疗提供有力的依据。     

鼻型NK/T细胞淋巴瘤26例临床病理分析

目的:研究鼻型NK/T细胞淋巴瘤(ENKL)的免疫表型、病理特点、临床特点。方法:回顾性分析26例ENKL的临床表现、病理组织学特点,采用免疫组化SP法检测LCA、CD3、UCHL1、CD20、CD79a、CD56、TIA-1、Granzyme B、perforin,原位杂交方法检测EBER在ENKL中的表达情况。结果:NK/T细胞淋巴瘤发生在鼻腔占80.77%(21/26),伴坏死、溃疡、鼻出血者100%(26/26),肿瘤细胞嗜血管现象占46.15%(12/26),伴"鳞状细胞癌样反应"占20.08%(6/26)。CD3、CD56、TIA-1、Granzyme B、perforin及EBER阳性表达率达100%。结论:临床及病理形态复杂多样性是NK/T细胞淋巴瘤的特点。病理诊断中须注意与炎症或高分化鳞状细胞癌鉴别。ENKL根据典型的临床表现、病理形态学改变、免疫表型特点及EBER原位杂交阳性能准确诊断。     

鼻腔人免疫缺陷病毒相关浆母细胞淋巴瘤临床病理分析

目的：探讨鼻腔人免疫缺陷病毒相关浆母细胞淋巴瘤的临床病理特点、诊断及鉴别诊断。方法应用光学显微镜观察、免疫组织化学染色、原位杂交法对1例人免疫缺陷病毒阳性原发鼻腔的浆母细胞淋巴瘤（PBL）患者肿瘤组织进行临床病理学检查,并复习相关文献。结果肿瘤组织弥漫性浸润,肿瘤细胞较大,圆形或椭圆形,胞质丰富,细胞核偏位,可见1～2个核仁,核分裂象易见,可见凋亡小体及星空现象。肿瘤细胞CD138、VS38c、CD79a弥漫阳性,CD20、EMA部分阳性,Ki-67阳性指数80%,κ轻链基因单克隆性重排,原位杂交检测EB病毒编码mRNA（EBER）阳性。结论原发鼻腔的浆母细胞淋巴瘤是一种罕见的弥漫增生的恶性肿瘤,肿瘤细胞形态类似B免疫母细胞,但是肿瘤细胞具有浆细胞的免疫表型。     

阑尾原发性弥漫大B细胞淋巴瘤一例并文献复习

  目的　探讨阑尾弥漫大B细胞淋巴瘤（DLBCL）的临床病理特点。方法　回顾性分析收治的阑尾原发DLBCL 1例,对阑尾切除标本进行光学显微镜观察,免疫组织化学染色,并复习文献。结果　光学显微镜下见阑尾结构破坏,肿瘤组织弥漫分布,细胞呈大中心细胞样,形态介于中心母细胞和中心细胞之间。免疫组织化学染色肿瘤细胞CD20、bcl-6、CD10、CD79α及Mum1阳性,Ki-67约90 %,CK、bcl-2、CD21及ALK均阴性。结论　阑尾DLBCL少见,结合组织学形态和免疫组织化学染色有助于明确诊断及判断预后。     

miRNA-200a在EB病毒感染相关的儿童散发性伯基特淋巴瘤中的表达及其意义

目的 探讨miRNA-200a在EB病毒(EBV)阳性和阴性的儿童散发性伯基特淋巴瘤(BL)中的表达及其意义.方法 应用原位杂交对40例BL患儿肿瘤组织及13例慢性扁桃体炎患者扁桃体组织进行EBER检测,应用qRT-PCR方法对Raji和Ramos细胞株、40例BL患儿肿瘤组织及13例慢性扁桃体炎组织进行miRNA-200a检测.结果 40例BL组织中EBER阳性16例,阴性24例,13例扁桃体EBER均阴性;miRNA-200a在EBV阳性BL组织表达量为0.033±0.018,较EBV阴性BL组织(0.504±0.214)和扁桃体组织(0.284±0.153)降低,差异有统计学意义(均P＜0.05);Raji和Ramos比较,miRNA-200a表达显著降低(P=0.000).结论 miRNA-200a的低表达与EBV阳性儿童散发性BL的发病机制密切相关,miRNA-200a可能起着类似于抑癌基因的作用.     

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.     

天津地区上呼吸道恶性淋巴瘤临床病理、免疫表型及其与EB病毒相关性的研究

目的 :研究天津地区上呼吸道恶性淋巴瘤的临床病理、免疫表型及与 EB病毒的关系。方法 :应用单克隆抗体 U CHL - 1、CD3、L2 6 、4KB5 、CD79a、L CA、EMA、溶菌酶等抗体及 CS1- 4(L MP- 1)进行免疫组化染色 ,以进行淋巴瘤确诊、淋巴瘤免疫表型研究和 EB病毒感染情况的分析 ;EB病毒寡核苷酸探针 (EBER1/ 2 )原位杂交检测 ,探讨瘤细胞 EB病毒感染情况及与 EB病毒潜在膜蛋白表达的相关性。结果 :12 5例上呼吸道原诊断为恶性淋巴瘤和高度怀疑恶性淋巴瘤的病例中 112例确诊为淋巴瘤 ,诊断符合率 89.6 %。其中 6 6例为 T细胞性淋巴瘤 (占5 8.93% ) ,46例为 B细胞性淋巴瘤 (占 41.0 7% )。男性明显多于女性 (85例 / 2 7例 )。平均年龄 48.2 7岁 (10岁～ 88岁 )。 EBV- EBER1/ 2原位杂交检测 T细胞性淋巴瘤 34例 ,17例阳性 ,占 5 0 % ,阳性细胞占肿瘤细胞的 2 0 %～80 % ,EBV潜在膜蛋白 L MP- 1检测 5 3例 ,11例阳性 ,占 2 0 .75 % ,阳性率低于 EBER探针。结论 :天津地区上呼吸道恶性淋巴瘤以 T细胞性淋巴瘤为多见 ,发病可能与 EB病毒感染有关。     

鼻型NK/T细胞淋巴瘤26例临床病理分析

目的：研究鼻型NK/T细胞淋巴瘤（ENKL）的免疫表型、病理特点、临床特点。方法：回顾性分析26例ENKL的临床表现、病理组织学特点,采用免疫组化SP法检测LCA、CD3、UCHL1、CD20、CD79a、CD56、TIA-1、Granzyme B、perforin,原位杂交方法检测EBER在ENKL中的表达情况。结果：NK/T细胞淋巴瘤发生在鼻腔占80.77%（21/26）,伴坏死、溃疡、鼻出血者100%（26/26）,肿瘤细胞嗜血管现象占46.15%（12/26）,伴＂鳞状细胞癌样反应＂占20.08%（6/26）。CD3、CD56、TIA-1、Granzyme B、perforin及EBER阳性表达率达100%。结论：临床及病理形态复杂多样性是NK/T细胞淋巴瘤的特点。病理诊断中须注意与炎症或高分化鳞状细胞癌鉴别。ENKL根据典型的临床表现、病理形态学改变、免疫表型特点及EBER原位杂交阳性能准确诊断。     

鼻NK/T细胞淋巴瘤的免疫表型、EBV感染及TCRγ基因重排的检测

 目的 检测鼻NK/T细胞淋巴瘤（NK/TCL）的免疫表型、EBV感染及TCRγ基因重排,为诊断和鉴别诊断提供依据。方法 收集诊断鼻NK/TCL48例患者石蜡包埋标本,用免疫组化SP法标记LCA、CD79α、CD20、CD56、CD3、CD45RO及EBV抗体研究其免疫表型;EBER探针原位杂交方法检测EBV编码的小分子RNA（EBER）;聚合酶链式反应扩增方法检测TCRγ基因重排。结果 48例鼻NK/TCL均表达LCA,CD3、CD45RO、CD56和EBV阳性率分别为44%、52%、73%和19%,CD79α和CD20均阴性;EBER阳性率为81%;TCRγ基因重排阳性率为19%。结论 鼻NK/TCL免疫表型不一致,并非所有病例CD56阳性,石蜡切片中CD3阳性定位于细胞质;EBER在肿瘤细胞中高表达,提示它们可能为NK细胞来源;部分TCRγ基因重排阳性病例应为鼻NK样T细胞淋巴瘤。     

Primary Testicular NK/T-Cell Lymphoma：A Study of Two Cases and Review of Literature

Primary testicular NK/T-cell lymphoma is an extremely rare entity progressed rapidly. The aim of this study was to examine clinical and pathological features of primary testicular NK/T-cell lymphoma and to investigate the effective diagnosis and prognosis. In this paper, the two cases of primary testicular NK/T-cell lymphoma were observed by light microscopy, immunohistochemistry and examined by in situ hybridization for Epstein-Barr Virus (EBV) DNA and the literatures were reviewed. The two patients respectively present with bilateral and right-side painless testicular enlargement. The morphology of neoplastic cells of case 1 were small to medium, tumor cells of case 2 were small, medium and large mixed. The tumor cells grew diffusely with irregular and distort nuclear, destructed the organizational structure of the normal testis, and damaged blood vessels, meanwhile, coagulation necrosis was exist. Immunohistochemical staining of neoplastic cells showed positive for CD45, CD2, CD56, CD3ɛ (cytoplasm staining pattern), CD45RO and Granzyme B, and negative for CD57, CD20, CD79α, CD30, CK, MPO, TdT, Bcl-2 and PLAP were negative. In addition, the EBV DNA was detected in the lymphoma by In situ hybridization. In conclusion, the expression of CD56, CD3ɛ, and Granzyme B associated proteins and EBV examination by in situ hybridization play a vital role in diagnosis and differential diagnosis of primary testicular NK/T-cell lymphoma.     

阑尾原发性腺癌晚期形成Krukenberg瘤的临床分析

目的:探讨阑尾原发性腺癌晚期形成Krukenberg瘤的临床特点、诊断及治疗。方法:分析1例表现为Krukenberg瘤的阑尾原发性腺癌患者的临床资料,并复习相关文献。结果:阑尾原发性腺癌晚期转移形成Krukenberg瘤的病例较为罕见,并且缺乏特异性临床表现,极易漏诊、误诊,术前或术中即明确诊断者极少见,绝大多数是通过术后组织病理学诊断而最终确诊。右半结肠切除术是阑尾原发性腺癌的首选治疗方式,术后辅以化疗等综合治疗。结论:以Krukenberg瘤为首发表现的病例在寻找原发癌灶时应考虑到原发性阑尾恶性肿瘤的可能。术中对阑尾的仔细探查是必要的,若发现阑尾形态异常改变除了及时行阑尾切除,术中还应对其进行冰冻病理检查以提高术中确诊率,指导手术治疗。     

Primary CD56 positive lymphomas of the gastrointestinal tract

BACKGROUND: Primary CD56 positive lymphoma of the gastrointestinal (GI) tract is rare. Genotypically, these tumors can be classified into natural killer (NK)-like T-cell lymphoma or NK cell lymphoma by the presence or absence of T-cell receptor (TCR) gene rearrangement. However, they have a considerable degree of morphologic and immunophenotypic overlap, making a definitive diagnosis difficult. METHODS: The clinicopathologic features of three patients with primary CD56 positive lymphoma of the small and large bowel are presented. This is followed by a review of the English literature from 1966 to the present. RESULTS: All patients had CD56 positive/CD3epsilon positive disease on paraffin section. Two patients were positive for Epstein-Barr virus-encoded early nuclear RNAs (EBER) according to in situ histochemistry results and were negative for TCR gene rearrangement, consistent with primary NK lymphoma of the GI tract. The other patient was EBER negative with rearranged TCR, consistent with NK-like T-cell lymphoma. There was no clinical or histologic evidence of enteropathy in any of the patients. The major presenting symptoms included fever, weight loss, and intestinal perforation. All patients died between 1 week and 6 months after diagnosis despite undergoing surgery and intensive chemotherapy. CONCLUSIONS: These results, together with a literature review, suggest that primary NK cell lymphoma of the GI tract may be considered a distinct clinicopathologic entity. Both primary NK and NK-like T-cell lymphoma pursue an aggressive clinical course. EBER and TCR gene rearrangement are useful in distinguishing NK cell lymphoma from NK-like T-cell lymphoma, particularly when frozen tissue is not available for immunophenotyping.     

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。     

Epstein-Barr Virus-Associated Nodal Malignant Lymphoma in Thailand

Specific subtypes of malignant lymphoma are highly associated with Epstein-Barr virus (EBV) infection. In the ‍present study, the authors evaluated EBV-encoded RNA (EBER) expression by in situ hybridization in 300 cases of ‍malignant lymphomas diagnosed by lymph node biopsy, with 100 cases of reactive lymphoid hyperplasia in lymph ‍nodes as controls, for comparison. There were 100 consecutive cases of classical Hodgkin’s lymphoma (cHL), 100 ‍consecutive cases of non-Hodgkin’s lymphoma, B cell (NHL-B), and 100 consecutive cases of non-Hodgkin’s ‍lymphoma, T cell (NHL-T). EBER expression was detected in 46% of reactive lymphoid hyperplasia cases, but the ‍positively stained cells in those cases constituted less than 5 percent of the total cell populations. When using the ‍presence of EBER in 5 percent or more of the cell population and/or the presence of EBER in the Hodgkin’s Reed- ‍Sternberg’s cells as indicators of positivity, 64% of cHL, 13% of NHL-B, and 51% of NHL-T were found to be ‍positive. The study indicates a strong association of cHL and NHL-T with EBV infection, the link apparently being ‍weaker for NHL-B except for the subtypes of Burkitt’s lymphoma and diffuse large B cell lymphoma. ‍     

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。     

CD5-positive, cyclinD1-negative mantle cell lymphoma with a translocation involving the CCND2 gene and the IGL locus

Distinguishing mantle cell lymphoma (MCL), from low-grade B-cell lymphoma is important because MCL is clinically more aggressive and is treated differently. Though most MCL overexpress cyclinD1 (CCND1) and have a t(11;14)(q13;q32), MCL that are negative for CCND1 exist. Some have translocations involving cyclinD2 (CCND2) and either the immunoglobulin heavy chain or kappa light chain locus. We present a CD5-positive, CCND1-negative B-cell lymphoma with a novel translocation involving CCND2 and the immunoglobulin lambda (IGL) gene. A 64-year-old male underwent resection of a polypoid mass of the ileum. Histology showed atypical, medium-sized lymphoid cells positive for CD20, CD5, CD43, and CCND2 by immunohistochemistry, and negative for CCND1, CCND3, and p27. Fluorescence in situ hybridization was negative for CCND1 abnormalities, but demonstrated a CCND2/IGL fusion. Clinical workup revealed stage IV disease. Current diagnostic criteria are insufficient for subclassifying this case, highlighting the need for additional studies on CCND2-translocated B-cell lymphomas to guide therapy appropriately.     

Cytologic diagnosis of Burkitt lymphoma

BACKGROUND: The diagnosis and classification of lymphoma require correlation of morphologic, immunophenotypic, and molecular-cytogenetic studies. Fine-needle aspiration biopsy (FNAB) is a valuable diagnostic technique that allows material to be collected for these ancillary studies, and for morphologic evaluation. METHODS: The authors report a series of seven cases clinically or morphologically suspicious for Burkitt lymphoma. Fluorescence in situ hybridization studies (FISH) for c-myc were performed on FNAB material and correlated with cytologic and immunophenotypic data. RESULTS: Six of seven specimens were positive for c-myc rearrangement by FISH. However, only three of these cases represented Burkitt lymphoma, with one additional case of atypical Burkitt lymphoma. The other cases included diffuse large B-cell lymphoma, monomorphic posttransplant B-cell lymphoma, and an aggressive B-cell lymphoma, with the latter case negative for c-myc rearrangement by FISH. Of 2 non-Burkitt lymphoma specimens tested, 1 was positive for the immunoglobulin H/bcl-2 rearrangement, in addition to the c-myc rearrangement, suggesting transformation from a lower grade lymphoma. CONCLUSIONS: These cases illustrated the value of FNAB in the diagnosis of Burkitt lymphoma, as well as the importance of obtaining material for, and integrating results of, ancillary studies for the final diagnosis.     

Burkitt transformation of mantle cell lymphoma

The associated poor prognosis and potentially aggressive behavior of mantle cell lymphoma and its blastoid variants make differentiation from other non-Hodgkin B-cell lymphomas especially important. We present a case of mantle cell lymphoma with a marked leukemic component, which demonstrated both a typical nodular mantle cell pattern and Burkitt lymphoma within a single lymph node removed at the time of splenectomy. The presence of CD5, CD10, and Bcl-1 co-expression by immunohistochemistry and detectable t(11;14) and cMYC gene rearrangement by FISH analyses in the Burkitt region support a transformation of mantle cell lymphoma over a concomitant malignancy. A limited number of mantle cell lymphomas demonstrating dual t(11;14) and chromosome 8q24 cMYC gene rearrangements have been previously reported in the literature. They demonstrate an extremely aggressive course with a very poor prognosis. Although the accelerated terminal phase of this patient's clinical course mirrors these previous published cases; none have described the combined morphologic and immunophenotypic features of Burkitt lymphoma reported here. This case provides further support for the aggressive nature of these lymphomas and demonstrates the utility of flow cytometry, immunohistochemistry, and cytogenetic techniques in avoiding potential errors in their diagnosis, prognosis, and treatment.