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1.
对1例皮肤的结外鼻型NK/T细胞淋巴瘤临床病理结合免疫组化染色、EB病毒原位杂交及T细胞受体基因重排进行分析.右胫后多发结节,组织病理特征为肿瘤组织在真皮及皮下组织内弥漫性浸润,肿瘤组织具有血管中心性及血管破坏性特点,肿瘤细胞具有异型性.瘤细胞表达CD2,CD56,颗粒酶B,EBER阳性,未检测到TCR克隆性基因重排.诊断为皮肤的结外鼻型NK/T细胞淋巴瘤.皮肤的结外鼻型NK/T细胞淋巴瘤恶性度高、预后差;诊断有赖于组织病理及免疫表型检测及EBER原位杂交技术.  相似文献   

2.
目的:报道1例皮肤结外鼻型NK/T细胞淋巴瘤,分析其临床表现、组织病理特点及治疗和预后,以提高皮肤科临床医生对本病的诊治水平.方法:通过临床表现、组织病理分析,结合免疫组化染色、EB病毒原位杂交确诊.结果:颈后皮损组织病理示真皮浅中层血管附属器周围几大灶淋巴样细胞浸润,细胞核大,胞浆透明,异型性明显.瘤细胞表达CD2、CD3、CD5、CD7、CD8、GranzymeB、Ki-67,而不表达CD56,EB病毒( + ).诊断为皮肤的结外鼻型NK/T细胞淋巴瘤.结论:结外鼻型NK/T细胞淋巴瘤具有独特的组织病理及免疫组化特征,恶性度高、易误诊、预后差.  相似文献   

3.
患者男,60岁,藏族。全身斑块、结节及肿块6月,肿块破溃伴疼痛2月。皮损病理组织示:表皮变薄,基底层液化变性,瘤细胞在真皮及皮下脂肪层呈弥漫性浸润,瘤细胞形态不一,核深染,可见血管中心性浸润及凝固性坏死。免疫组化标记瘤细胞CD3,CD3ε,CD56,TIA-1,粒酶B均为(+),CD30约10%(+),CD8(-),CD79a(-),EBER1/2原位杂交(+)。TCR-γ重排未见明确克隆性重排条带。诊断:皮肤结外鼻型NK/T细胞淋巴瘤。  相似文献   

4.
皮肤NK/T细胞淋巴瘤的临床病理及其与EB病毒关系的研究   总被引:6,自引:0,他引:6  
目的探讨皮肤 NK/T细胞淋巴瘤的临床病理特点、免疫表型及与 EB病毒感染的关系。方法应用免疫组织化学染色,选用 CD45RO、 CD3ε、 TIA- 1、 CD20、 Ki- B5、 CD68和 LMP1等抗体;用 EBER1/2原位杂交检测 EB病毒编码的小分子 RNA。结果 5例皮肤 NK/T细胞淋巴瘤患者占同期皮肤恶性淋巴瘤的 5.68%;男 4例,女 1例,平均年龄 34岁,主要表现为皮肤无症状肿块, 2例有溃疡形成;组织学特征为肿瘤在真皮和皮下脂肪内,易形成血管浸润性和破坏性病变,瘤细胞大小、形态各异;瘤细胞表达 T细胞标记( 5/5)和 NK细胞或细胞毒性 T细胞标记 TIA- 1( 4/4), EBER1/2( 3/4)。结论皮肤 NK/T细胞淋巴瘤具有临床病理独特性,与 EB病毒感染有较强的相关性。  相似文献   

5.
血管内NK/T细胞淋巴瘤(intravascular NK/T?cell lymphoma,IVNKTL)是一种具有NK/T细胞免疫表型的血管内淋巴瘤,临床罕见。IVNKTL与EB病毒感染相关,临床呈高度侵袭性,与另一种EB病毒相关的结外NK/T细胞淋巴瘤,鼻型(extranodal NK/T?cell lymphoma,nasal type,ENKTCL)相似……  相似文献   

6.
目的:探讨原发皮肤结外NK/T细胞淋巴瘤-鼻型(EN-NK/T-NT)的临床、组织病理学、免疫表型及基因型特点.方法:对2例原发皮肤EN-NK/T-NT进行临床特征、组织病理学形态和免疫组化分析,以及EB(Ebstein-Barr)病毒(EBV)原位杂交及T细胞受体γ基因PCR重排检测.结果:2例患者发病均表现为皮肤症状,双下肢皮肤多发浸润性红斑伴溃疡,无中面部症状.组织病理均示中至大型异形淋巴细胞弥漫浸润真皮和皮下组织,肿瘤细胞核不规则,核仁不明显,胞质中等量,可见血管壁浸润,凋亡及坏死明显.免疫组化肿瘤细胞CD3ε、CD56、细胞毒蛋白、表达于T细胞的T-box(T-bet)及erythroblastosis virus E26oncogene homolog(ETS)-1阳性,皮肤淋巴细胞相关抗原(CLA)例1阴性、例2阳性;EBV强阳性;T细胞受体γ基因重排呈胚系构型.结论:原发皮肤EN-NK/T-NT与原发鼻EN-NK/T-NT组织病理学及相关分子生物学特点一致,其发生与发展转归值得深入研究.  相似文献   

7.
目的 探讨生存素、Ki67在皮肤结外鼻型NK/T细胞淋巴瘤中的表达及意义.方法 选取确诊的以皮损为首发表现的15例皮肤结外鼻型NK/T细胞淋巴瘤为研究对象,常规SP免疫组化染色法检测生存素、Ki67.每张切片选取5个有代表性的高倍镜视野,每个视野随机计数200个肿瘤细胞,分别计算1000个肿瘤细胞内生存素、Ki67阳性细胞百分比.结果 15例皮肤结外鼻型NK/T细胞淋巴瘤中,9例瘤细胞表达CD56,13例表达CD3ε,15例表达TIA-1,10例表达粒酶B,2例表达CD3.15例均表达1~2个T细胞抗原(CD2、CD45RO或CD7),1例表达βF1,3例表达CD30的病例阳性细胞均为大瘤细胞.所有病例均不表达CD4、CD5、CD8、CD20和CD79α.14例中3例被检出TCR-γ基因克隆性重排,15例EBER1/2原位杂交均阳性.15例中,11例(73.3%)表达生存素,阳性细胞表达率为23.97%±18.35%;14例(15例中1例掉片)均表达Ki67,阳性细胞表达率41.20%±19.52%.核分裂0~2个/高倍视野的9例Ki67表达阳性细胞率为25.27%±12.96%,核分裂>2个/高倍视野的6例为58.23%±16.02%,两组差异有统计学意义(F=19.14,P=0.001).皮肤结外鼻型NK/T细胞淋巴瘤中生存素、Ki67都有较高的表达,生存素与Ki67表达之间无相关性.结论 生存素、Ki67的高表达可能在皮肤结外鼻型NK/T细胞淋巴瘤发生发展中起着一定作用.  相似文献   

8.
患者女,38岁。全身红斑、坏死、溃疡伴疼痛发热十月余,加重1个月。皮损表现为小腿部位多发红斑、结节、溃疡及坏死,多次行皮损组织病理检查误诊为"坏疽性脓皮病"。1个月前皮损累及面部,再次行病理活检示:真皮小血管及神经纤维周围伴多量淋巴样细胞浸润,皮下组织并见脂膜炎反应。免疫组化:CD2(+),CD3(+),TIA-1(+),Gr B(+),CD56(+),Ki67[约90%+]及合并EBER(+);TCR无克隆性基因重排。诊断:结外NK/T细胞淋巴瘤(鼻型)。  相似文献   

9.
74岁女性患者,双下肢反复多发肿块伴溃疡6个月。皮损组织病理示:大、中、小3种肿瘤细胞混合浸润,有明显的血管中心性和血管破坏,伴广泛的凝固性坏死和溃疡。免疫表型:CD3ε、CD43、CD56、TIA-1、EB病毒潜伏膜蛋白(EBV LMP-1)、粒酶B(Granzyme B,Gr B)均阳性,Ki-67阳性(≥85%),CD8+CD4+。全身检查未发现皮肤以外系统受累证据,诊断为原发皮肤结外NK/T细胞淋巴瘤-鼻型。该病恶性程度高,需尽早进行组织病理检查和免疫组化染色以帮助诊断。  相似文献   

10.
目的 报道6例牛痘样水疱病样皮肤淋巴瘤,并研究其与慢性活动性EB病毒感染的关系。方法 临床病理分析、皮损免疫组织化学染色、血清学分析、EB病毒编码RNA原位杂交、外周血EB病毒DNA测定。结果 6例患者皮损均为反复发作的丘疹、丘疱疹、坏死、痘疮样瘢痕,其中4例还伴有程度不同的颜面、手足水肿。所有患儿均有长期间断发热等症状。皮损病理可见表皮多房性水疱,真皮全层大量淋巴细胞浸润,细胞形态异形,可见病理分裂象。4例皮损病理免疫组化染色,可见大量CD56阳性细胞,散在的CD3和CD45RO阳性细胞,T细胞内抗原-1和粒酶B染色阳性,诊断为牛痘样水疱病样皮肤NK/T细胞淋巴瘤;2例组化染色CD3和CD45RO阳性,CD56阴性,诊断为牛痘样水疱病样皮肤T细胞淋巴瘤。6例皮损均可见EB病毒编码RNA原位杂交阳性肿瘤细胞,血清学检查EB病毒衣壳抗原IgG抗体滴度升高,其中2例滴度为1 ∶ 5120,2例为1 ∶ 2560,2例为1 ∶ 1280;2例患者外周血EB病毒DNA拷贝数高于正常。6例患儿均证实患有慢性活动性EB病毒感染。结论 牛痘样水疱病样皮肤淋巴瘤主要表现为颜面手足肿胀、水疱、痘疮样瘢痕,病理表现主要为真皮异形淋巴细胞浸润和血管中心坏死,免疫表型以NK/T型多见。慢性活动性EB病毒感染与该型淋巴瘤发病密切相关。  相似文献   

11.
Cutaneous peripheral T-cell lymphoma unspecified is a rare neoplasm that is infrequently associated with Epstein-Barr virus (EBV) infection. In contrast, extranodal natural killer (NK)/T-cell lymphoma, although also rare, is known to be strongly associated with EBV and occurs most commonly in the nasal region. We report the case of a 55-year-old male who presented with fever and an indurated cutaneous plaque with ulceration. This cutaneous neoplasm showed diffuse dermal lymphomatous infiltration and tumor necrosis, with neoplastic cells expressing CD2, cytoplasmic CD3 (CD3ε), CD8, CD16, CD30, T-cell intracellular antigen-1, and granzyme B but not CD56, BF1, or T-cell receptor (TCR) δ1. Furthermore, the tumor cells were noted to be diffusely positive for EBV by in situ hybridization. A monoclonal TCR gene rearrangement was demonstrated. The disease showed an aggressive clinical course, and the patient died within 3 weeks of diagnosis without complete staging or chemotherapy. According to the 2005 World Health Organization/European Organization for Research and Treatment of Cancer scheme for cutaneous lymphoma and the 2008 WHO classification for lymphoid neoplasms, our case would have been classified as a nasal type extranodal NK/T-cell lymphoma with T-cell lineage. However, the expressions of CD8 and CD16, in addition to a monoclonal TCR gene rearrangement, are unusual findings in NK/T-cell lymphoma, and we believe such a phenotype/genotype should be more appropriately classified as an EBV-positive peripheral T-cell lymphoma, unspecified with a cytotoxic phenotype. Detailed clinicopathologic and molecular studies of similar cases may shed light on the prognostic impact of NK vs. T-cell lineage on extranodal NK/T-cell lymphomas.  相似文献   

12.
报道1例原发皮肤结外NK/T细胞淋巴瘤,并复习文献。患者,女,42岁。全身皮肤瘀斑、皮下结节20余天,发热4 d。右股内侧皮损组织病理示:大量淋巴细胞及浆细胞呈弥漫性浸润。免疫组化结果:CD3、CD43、CD56、颗粒酶B(Granzyme B,GгB)、细胞毒性蛋白(TIA)-1均(+)、Ki-67 LI约60%阳性;原位杂交EBER(+)。本病恶性程度高,需尽早进行组织病理检查及免疫组化染色以帮助诊断。  相似文献   

13.
We report a 51-year-old woman with cutaneous involvement by extranodal NK/T-cell lymphoma (TCL) of the colon that microscopically mimicked mycosis fungoides (MF). She had a history of fever of unknown origin for 2 months and then developed multiple erythematous papules on her trunk and extremities. A skin biopsy revealed superficial infiltration by atypical small to medium-sized lymphocytes with epidermotropism and Pautrier collections. Immunohistochemical studies showed expression of CD3 and TIA-1 with lack of expression (double negative) of CD4 and CD8. Initially, we reported the diagnosis as MF, cytotoxic variant. Thereafter, computerized tomography scan incidentally identified a colonic mass. A colonic biopsy revealed infiltration of atypical lymphoid cells with the same morphology and immunophenotype as those found in the skin. Additionally, CD56 and Epstein-Barr virus-encoded RNA in situ hybridization in both skin and colonic biopsies were diffusely positive. Thus, extranodal NK/TCL was diagnosed. Delta T-cell receptor (TCR) gene rearrangement was documented in the skin biopsy by polyacrylamide gel electrophoresis and fluorescence capillary gel electrophoresis methods. There was no TCR gene rearrangement detected in the colonic biopsy. Unfortunately, the patient died within 2 months of diagnosis.  相似文献   

14.
We present an unusual case of a CD56-positive T-cell lymphoma exhibiting immunophenotypic characteristics of both γδ T-cell lymphoma and extranodal NK/T-cell lymphoma, nasal-type. The patient presented with a 2-month history of rapidly progressive, pruritic and cutaneous nodules on his arms. A biopsy showed a dense pan-dermal infiltrate of markedly atypical CD3-positive lymphocytes, compatible with tumor stage cutaneous T-cell lymphoma. Retrospective review of a preceding biopsy and flow cytometric analysis, performed at an outside institution, showed strong expression of surface CD3, CD7, CD43 and γδ T-cell receptor (TCR), findings consistent with a diagnosis of cutaneous γδ T-cell lymphoma. In light of these data, we performed additional studies that showed diffuse positive staining of the atypical lymphocytes for CD56, CD4 and CD43 as well as Epstein-Barr virus-encoded small nonpolyadenylated RNA (EBER). Interestingly, this case displays characteristic features of γδ T-cell lymphoma, with strong surface expression of CD3 and γδ-TCR, as well as characteristics of natural killer (NK)/T-cell lymphoma, including expression of CD4 and EBER positivity, that represent two separate categories in the current classification of cutaneous lymphomas. Taken together, these findings underscore the difficulty of rendering an unambiguous classification of the presented neoplasm given the close ontogenetic relationship between NK and cytotoxic T-cells and highlight the need for continued reevaluation of the current classification system.  相似文献   

15.
Natural killer (NK)/cytotoxic T-cell lymphoma, a new type of cutaneous neoplasm, has been described recently in the World Health Organization/European Organization for Research and Treatment of Cancer classification for cutaneous lymphomas. We report an 11-year-old boy who had had erythematous plaques and blisters on his face and hands for 4 years and infiltrating plaques and necrosis on his extremities for 4 months. Routine clinical and laboratory examinations found no primary nasal involvement. Biopsies taken from nasal mucosa and skin showed that the tumour only involved dermis and subcutaneous tissue, and the infiltrated lymphohistiocytic tumour cells were CD56+, TIA+, CD45RO+ and CD30+. In situ hybridization for EBV-encoded nuclear RNA was positive. Clonal T-cell receptor-gamma2 gene rearrangement was positive. A diagnosis of extranodal NK/T-cell lymphoma, nasal type, was made. This is a rare case, with slow course and survival for >51 months with the presentation only occurring in the skin.  相似文献   

16.
目的报道1例原发皮肤的儿童结外NK/T细胞淋巴瘤,鼻外型,并回顾文献,学习该病的临床特征、组织病理、免疫组化及治疗预后特点,以提高临床医生对该病的认识。方法分析本例原发皮肤的儿童结外NK/T细胞淋巴瘤-鼻外型患者的临床表现、辅助检查、病理组化及治疗预后,并复习近年国内外相关文献。结果 11岁男性患儿表现为皮肤结节、斑块伴乏力,肝脾淋巴结明显肿大。实验室检查示白细胞和血小板降低,肝酶升高,乳酸脱氢酶升高,凝血时间延长,血液EBV-PCR高度复制。组织病理提示局部或弥漫大小不一淋巴细胞浸润,可见核碎裂,部分细胞核大深染。免疫组化:CD3(+)、CD20(-)、CD56(+++)、颗粒酶B(+)、T细胞胞浆内抗原-1(+)、Ki-67约30%~40%(+);EB病毒编码RNA原位杂交(+++)。诊断:原发皮肤的结外NK/T细胞淋巴瘤,鼻外型。告知病情后家属放弃治疗。结论不同于鼻型,本病的临床表现特异性不高,易误诊为脂膜炎等,多合并系统受累,确诊依靠组织病理、免疫组化、EBV病原学检验及结合临床表现。尽管采用强势化疗,本病仍预后不良。  相似文献   

17.
In the CD56+ cutaneous nasal-type NK/T-cell lymphoma strongly associated with latent EBV infection, subcutaneous or dermal nodules are the most common skin findings, but great morphologic heterogeneity has been noted including papules, infiltrated plaques, and ulcerated tumors, and TCR genes are mostly germline. We describe a case of nasal and nasal-type NK/T-cell lymphoma featuring multiple erythematous polycyclic patches on the trunk, which is similar to patch stage mycosis fungoides or other cutaneous T cell lymphoma. Immunohistochemical study of a skin biopsy specimen revealed CD2+, CD3epsilon+, CD56+, and CD45RO+ expression in the neoplastic cells. In situ hybridization using an anti-sense Epstein Barr virus early regions probe showed a positive reaction. However, clonal TCR beta gene rearrangement was found.  相似文献   

18.
BACKGROUND: Some lymphomas express natural killer (NK)-cell markers such as the neural cell adhesion molecule, which is recognized by the CD56 antibody. These lymphomas may present in the skin, but do not represent a homogeneous group. The new World Health Organization classification of lymphoma/leukaemia recognizes several types of NK/T-cell neoplasm, including blastic NK-cell lymphoma, which characteristically presents with cutaneous lesions. OBJECTIVES: To describe the clinical, pathological and molecular features in six cases of CD56+ lymphoma with cutaneous presentation. METHODS: The clinical, histopathological and immunophenotypic features of six patients were reviewed. In addition, in situ hybridization (ISH) to identify Epstein-Barr virus (EBV) mRNA, and polymerase chain reaction analysis to identify the presence of a clonal population of T cells or B cells were performed on lesional skin. RESULTS: All patients presented with widespread nodules and plaques, which in five cases were a characteristic purple colour. Four patients developed disseminated disease, three with neurological involvement. These four patients died between 14 and 46 months following diagnosis (median 30 months). In four of six cases the histopathological and immunohistological features were in keeping with a blastic NK-cell lymphoma. No clonal immunoglobulin heavy chain (IgH) or T-cell receptor (TCR) gene rearrangement was detected in the four cases consistent with an origin from NK cells. A further case fitted the criteria for an extranodal NK/T-cell lymphoma of nasal type and was also the only case to show evidence of EBV mRNA by ISH. A clonal T-cell population was identified in the final case. This patient also exhibited molecular evidence of a clonal B-cell population and a t(14;18) translocation confirmed by sequence analysis. CONCLUSIONS: Our data confirm that NK-cell lymphomas presenting in the skin are a heterogeneous group, and that in the U.K., blastic NK-cell lymphoma is more common than extranodal NK/T-cell lymphoma of nasal type. These lymphomas pursue an aggressive course, with rapid development of disseminated disease, and resistance to chemotherapy. Detailed immunophenotyping is needed to distinguish the different types. Our molecular data indicate that blastic NK-cell lymphoma cases lack clonal TCR/IgH gene rearrangements consistent with an NK-cell origin. Our ISH findings indicate that EBV plays a pathogenetic role only in extranodal NK/T-cell lymphoma of nasal type.  相似文献   

19.
Primary cutaneous γδ T‐cell lymphoma and extranodal natural killer (NK)/T‐cell lymphoma (ENKTL), nasal type are two distinct lymphoma entities in the World Health Organization (WHO) classification. We report the case of an aggressive cutaneous lymphoma of γδ T‐cell origin showing overlapping features of both lymphomas. A 78‐year‐old female presented with confluent erythematous plaques with ulcerations over her right thigh. Microscopically, section of the skin showed a diffuse dermal and subcutaneous lymphocytic infiltration with tumor necrosis and angioinvasion. The medium‐ to large‐sized tumor cells expressed CD3, CD8, cytotoxic molecules and T‐cell receptor (TCR)‐γ but not CD4, CD20, CD30, CD56 or βF1. In situ hybridization for Epstein‐Barr virus‐encoded mRNA (EBER) was diffusely positive. Polymerase chain reaction‐based clonality assay showed a clonal TCR‐γ chain gene rearrangement. The features compatible with γδ T‐cell lymphoma include dermal and subcutaneous involvements, cytotoxic phenotype, expression of TCR‐γ, as well as an aggressive course. On the other hand, the diffuse EBER positivity, angioinvasion, tumor necrosis and cytotoxic phenotype may also fit in the diagnosis of an ENKTL of T‐cell lineage. We review the literature on EBER‐positive γδ T‐cell lymphoma and discuss the diagnostic dilemma using the current WHO classification system.  相似文献   

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