首页 | 本学科首页   官方微博 | 高级检索  
相似文献
 共查询到15条相似文献,搜索用时 140 毫秒
1.
目的 探讨儿童皮下脂膜炎样T细胞淋巴瘤(SPTL)的临床病理、免疫表型、基因重排及EB病毒(EBV)感染情况。方法 将收集的5例儿童SPTL作临床病理和免疫组化分析,采用PCR检测TCRγ、IgH基因重排,运用EBER1/2原位杂交检测EBV感染。结果 男4例、女1例,年龄9 ~ 13岁,主要表现为无症状的结节、斑块或肿块。组织学上肿瘤在皮下脂肪内呈脂膜炎样浸润,细胞大小不等、异形性明显,瘤内可见豆袋细胞、上皮样肉芽肿和小片状坏死。瘤细胞表达T细胞标记βF1、CD2、CD3、CD8、CD45RO和细胞毒颗粒相关蛋白标记TIA-1、粒酶B,不表达CD4、CD20。4例检出单克隆性TCRγ基因重排,未检出IgH基因重排。EBER1/2原位杂交阳性2例。获随访的3例中1例死于本病,该例EBER1/2阳性。结论 儿童SPTL面部易受累,全身症状多见,噬血细胞综合征发生率与死亡率较高。伴有EBV感染者可能预后较差。  相似文献   

2.
目的 分析皮下脂膜炎样T细胞淋巴瘤(SPTL)的临床及组织病理表现、免疫表型、治疗和预后.方法 回顾性分析9例SPTL患者的临床与实验室资料,完善相关病理及免疫组化标记,并随访.结果 9例患者中8例表现为多发皮下结节和肿块,主要累及下肢(8例)和躯干(6例),7例伴发热.3例行全身PET-CT,7例行骨髓穿刺,未见皮肤外内脏系统肿瘤,均不伴噬血细胞综合征.皮肤组织病理示异形单个核细胞核大、深染,主要浸润皮下脂肪组织,围绕单个脂肪细胞呈环状排列,真皮层附属器和血管周围可见肿瘤细胞浸润(5/9例).免疫表型示9例肿瘤细胞表达βF1、CD3、CD8,8例表达粒酶B和T细胞胞内抗原1(TIA-1),均不表达CD4、CD20、CD30、CD56.5例接受化疗(1例儿童和1例产后妇女),1例儿童接受甲泼尼龙冲击治疗,随访8例治疗后均达到临床完全缓解.结论 SPTL来源于α/βT细胞,组织病理和免疫组化检查有助于诊断及鉴别诊断.  相似文献   

3.
目的:研究皮下脂膜炎样T细胞淋巴瘤(SFTL)的临床病理特征、免疫表型、组织起源和预后.方法:按照2005年皮肤淋巴瘤世界卫生组织-欧洲癌症治疗研究组织(WHO-EORTC)新分类分析6例SPTL患者的临床资料.作常规组织病理检查和免疫组化标记,并对6例患者石蜡组织和1例患者冰冻新鲜组织切片进行15F1标记.结果:6例患者中男2例.女4例,中位年龄25.5岁.临床皮损以无痛性皮下结节和(或)斑块为主,少数患者有发热、贫血和脾大.瘤细胞主要限于皮下脂肪组织内,有异形性和核分裂,并见不同程度脂肪坏死和组织细胞吞噬现象.瘤细胞表达βF1、CD3、CD8、TIA-1、GB、LCA和CD45RO,不表达CD4、CD30、CD56、CD20和CD79a.6例SVTL患者平均随访37个月内均获缓解,仅1例在确诊42个月后因并发糖尿病、高血压及心衰而死亡.结论:SPTL是一种起源于αJβT淋巴细胞的罕见类型细胞毒性皮肤淋巴瘤,临床病程迁延反复,5年生存率高达80%以上.完整充分的免疫组化标记对SPTL的确诊和分型必不可少.  相似文献   

4.
皮下脂膜炎样T细胞淋巴瘤1例   总被引:2,自引:2,他引:0  
报告1例皮下脂膜炎样T细胞淋巴瘤。患者女,34岁。四肢出现疼痛性红斑、结节9年,伴有发热、消瘦等。组织病理学改变主要在皮下脂肪组织内,可见瘤细胞浸润于脂肪细胞间。免疫组化显示瘤细胞表达CD45Ro及CD68,不表达CD20。  相似文献   

5.
目的:研究儿童皮下脂膜炎样T细胞淋巴瘤(SPTCL)的临床和病理特点及与EB病毒感染的关系。方法:选用CD20、CD5RO.CD68.CD30、CD56、TIA—1等抗体作免疫组织化学ABC法染色;应用EBER1/2原位杂交检测EB病毒,结果:13例SPTCL占同期观察的皮肤非何杰金淋巴瘤的36.1%,其中男8例,女5例;发病年龄平均4.8岁;主要表现为下肢、躯干无症状性结节或肿块,常伴发热和肝脾肿大。组织学特点为肿瘤花皮下脂肪内呈脂膜炎样浸润,瘤细胞大小不等、形态各异,瘤内可见上皮样肉芽肿、多核巨细胞、豆袋细胞、小片坏死。免疫表型:TIA—1抗体表达12例,13例CD5RO均为阳性,CD20、CD30、CD56均为阴性;EB病毒EBER1/2原位杂交阳性率38.5%。13例中随访10例,死亡5例,其中4例合并噬血细胞综合征(HPS),4例中EBER阳性3例。结论:SPTCL在儿童皮肤非何杰金淋巴瘤中并不少见.儿童SPICL可能与EB病毒潜伏感染有一定的相关性。与EB病毒相关的SPTCL具有更大的侵袭性,常伴有HPS.预后较差。  相似文献   

6.
皮下脂膜炎样T细胞淋巴瘤17例临床组织病理特点研究   总被引:1,自引:0,他引:1  
目的观察皮下脂膜炎样T细胞淋巴瘤(SPTCL)临床及病理特点。方法用HE和免疫组化ABC法对17例SPTCL进行临床病理观察。结果17例病人主要表现为1cm×1cm~10cm×20cm的单个或多个结节、斑块和皮下包块,6例伴发热(体温呈中、高热)。组织学上主要病变均在皮下脂肪组织内,瘤细胞主要浸润于脂肪细胞之间。免疫组化显示所有病例瘤细胞均表达CD45RO或CD3、均不表达CD20及CD68。结论SPTCL是具独特临床病理特征的外周T细胞淋巴瘤,为皮肤原发淋巴瘤的新亚型。  相似文献   

7.
皮下脂膜炎样T细胞淋巴瘤研究进展   总被引:4,自引:0,他引:4  
皮下脂膜炎样T细胞淋巴瘤是近年新认识的少见的皮肤T细胞淋巴瘤,来源于αβTCRT细胞或γδTCRT细胞的细胞毒性T细胞。皮下脂膜炎样T细胞淋巴瘤主要表现为皮下多发性结节、斑块或皮下肿块,部分患者可伴有噬血细胞综合征,病情进展迅速。病理上可见皮下脂膜炎样改变,其中可见瘤细胞围绕单个脂肪细胞形成特征性的“花环样改变”,免疫组化分析为细胞毒性T细胞来源,表达αβTCR或γδTCR基因重排,对常规化疗反应欠佳,预后较差,其预后与其免疫表型有关。  相似文献   

8.
报告1例皮下脂膜炎样T细胞淋巴瘤。患者男,26岁。曾无防护地接触放射性元素钴3年。躯干部出现暗红色肿块1.5个月,伴有全血细胞减少。皮损组织病理学改变主要为真皮中下部及皮下脂肪层弥漫性瘤细胞浸润。免疫组化染色显示LCA(+)、CD45RO(+)、CD3(+)、CD2(+)、CD8(+)、TIA-1(+)、CD20(-)。  相似文献   

9.
皮下脂膜炎样T细胞淋巴瘤1例   总被引:3,自引:3,他引:0  
报道1例皮下脂膜炎样T细胞淋巴瘤。患者女,34岁。全身反复出现肿块、结节性损害1年。4个月前部分肿块破溃,伴发热、乏力、贫血等症状。皮损组织病理显示皮下脂肪层的淋巴细胞、组织细胞浸润,脂膜炎样T细胞瘤。免疫组化证明下细胞来源,伴有TCRγ基因克隆性重排,诊断为脂膜炎样T细胞瘤。  相似文献   

10.
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   

11.
目的探讨皮下脂膜炎样T细胞淋巴瘤(SPTL)中EB病毒(EBV)的潜伏感染情况及其与P53蛋白表达的关系。方法采用EBER1/2原位杂交检测20例SPTL组织中的EBV感染;用免疫组化LSAB法检测P53蛋白表达。结果20例SPTL中5例(25%)EBER1/2原位杂交阳性,6例(30%)的P53阳性。统计学分析,二者具有相关性(r= 0.533,P<0.05)。结论部分SPTL病例伴EBV潜伏感染,伴有EBV感染的SPTL患者可能预后较差。SPTL组织中P53蛋白表达可能与EBV的感染有关。  相似文献   

12.

Background

Subcutaneous panniculitis-like T-cell lymphoma (SPTL) is a distinctive skin lymphoma characterized by neoplastic T-cell infiltration of the subcutaneous tissue, mimicking panniculitis.

Objective

To describe the clinical and pathologic features of SPTL in Korean patients.

Methods

Fourteen SPTL patients evaluated over 15 years were retrospectively reviewed.

Results

The mean patient age was 35 years (range: 7~73 years), with male predominance (2.5:1). Most patients presented with either nodules or plaques, occurring most commonly on the trunk, with two patients (14%) having hemophagocytic syndrome. Histopathologically, all patients showed infiltrates of small-to-medium pleomorphic cells mimicking panniculitis, with some also showing rimming, bean-bag cells, and fat necrosis. Most patients were positive for CD3 (14/14), CD8 (12/13), TIA-1 (9/9) and βf1 (5/5), but were negative for CD4 (11/12), CD20 (8/8), CD56 (14/14) and Epstein-Barr virus (8/8). Ten patients (71%) received chemotherapy and 2 (14%) died due to the disease, with an average survival time of 4 months. Survival analysis did not reveal any significant prognostic factors.

Conclusion

This is the first series of patients with SPTL in Korea. Due to its indolent clinical course and relatively high survival rate, SPTL should be differentiated from cutaneous γδ T-cell lymphoma.  相似文献   

13.
Primary cutaneous γδ-T-cell lymphoma (CGD-TCL) is a rare entity of cutaneous T-cell lymphomas (CTCL) and is characterized by tumoral growth of mature γδ-T-cell expressing cytotoxic molecules. The prognosis of CGD-TCL is generally worse than other CTCL. However, relatively indolent patch/plaque lesions have been described suggesting the heterogeneous nature of this entity. Here, we present a case of CGD-TCL with various skin manifestations, such as erythematous plaques/tumors and subcutaneous panniculitis-like lesions. During the follow up, testicular involvement was detected, which was surgically removed. Histopathology showed mixed features from epidermotropism, dermal infiltration and subcutaneous panniculitis-like lesions depending on the clinical manifestations. The tumor cells were positive for CD3 and revealed cytotoxic markers, TIA-1 and perforin, but not for CD4, CD8, CD20, CD56, TCRβF1 or EBER. Topical glucocorticoid ointment, narrowband ultraviolet B (NB-UVB) irradiation and low-dose methotrexate (MTX) were effective to control these skin lesions. No visceral involvement was detected thereafter. While CGD-TCL is usually associated with poor prognosis, it seems to be composed of various clinical manifestations, and NB-UVB and low-dose MTX could be a choice for indolent patch/plaque and possibly nodular lesions, especially for the aged.  相似文献   

14.
原发皮肤 CD30阳性间变大细胞淋巴瘤七例临床病理分析   总被引:2,自引:0,他引:2  
目的:探讨原发皮肤 CD30阳性间变大细胞淋巴瘤(PC-ALCL)的临床与组织病理学特征。方法回顾性分析7例 PC-ALCL 患者的临床及病理资料情况。结果7例患者中,男6例,女1例,平均发病年龄52岁。皮损为红色结节、肿块和(或)斑块,3例多发,4例单发,6例伴溃疡形成。所有患者均未见系统受累。组织病理改变:肿瘤细胞在真皮内弥漫性分布,细胞体积大,胞质丰富,胞核呈异形改变,可见核分裂象。肿瘤细胞CD30和细胞毒蛋白阳性,CD20、CD56、间变性淋巴瘤激酶(ALK)和 EB 病毒编码小 RNA 原位杂交均阴性。结论 PC-ALCL 是一种少见的原发于皮肤的低度恶性 T 细胞淋巴瘤,结合临床表现、皮损组织病理及免疫组化检查可确诊,罕见系统受累及转移。  相似文献   

15.
BACKGROUND: Merkel cell carcinoma (MCC) of the skin is an aggressive but rare malignant neuroendocrine tumor. For its pathological diagnosis, we use a panel of immunohistochemical markers, such as cytokeratin 20 (CK 20), epithelial membrane antigen (EMA), chromogranin A, neuron specific enolase (NSE), synaptophysin, and Leu7 (CD57) to demonstrate its epithelial and neuroendocrine features. CD56, or neural cell adhesion molecule (NCAM), has been demonstrated recently as the tumor marker of the pulmonary neuroendocrine cell system. Its expression in MCC, however, has still rarely been investigated. Furthermore, in such very few previous studies on NCAM expression in MCC, all the tumor cells were not necessarily demonstrated to express NCAM. OBJECTIVES: To study the immunoreactivity of CD56 in MCC, especially using a monoclonal antibody of a clone 1B6, different from those adopted in the previous reports. METHODS: We reexamined CD56 expression immunohistochemically in five MCC cases, along with the conventional panel of markers described above, using paraffin-embedded tissue sections. RESULTS: CD56 revealed the most diffuse and intense positive staining, which was noted along the cell borders, in all specimens compared with other neuroendocrine tumor markers. CONCLUSIONS: The results of our study indicate that CD56, especially a new monoclonal antibody (clone 1B6), is a useful immunohistochemical marker for MCC.  相似文献   

设为首页 | 免责声明 | 关于勤云 | 加入收藏

Copyright©北京勤云科技发展有限公司  京ICP备09084417号