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1.
皮肤T细胞淋巴瘤是一组原发于皮肤的非霍奇金淋巴瘤,蕈样肉芽肿和Sézary综合征占绝大部分。肿瘤细胞早期有丝分裂指数较低及对细胞毒性药物的治疗反应差,提示细胞凋亡缺陷在其发病机制中起重要作用。本文以蕈样肉芽肿和Sézary综合征为重点,对凋亡在皮肤T细胞淋巴瘤发病与治疗中的作用进行了综述。  相似文献   

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随着对皮肤T细胞淋巴瘤(CTCL)病理发生机制研究的深入,靶向治疗及免疫调节治疗逐渐成为进展期CTCL的重要治疗手段。近年来与之相关的新的治疗方法不断涌现,如维A酸类X受体激动剂、融合毒素、单克隆抗体、Toll样受体激动剂、基因治疗、细胞因子等。与传统药物相比,靶向治疗及免疫调节治疗具有更显著的疗效及较好的安全性,临床应用前景良好。该文对其作一综述。  相似文献   

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阐述皮肤T细胞淋巴瘤向大细胞淋巴瘤转化的临床表现、组织病理、免疫组织化学染色、分子生物学特征、诊断和鉴别诊断与治疗。  相似文献   

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皮肤T细胞淋巴瘤分子生物学研究进展   总被引:1,自引:0,他引:1  
皮肤T细胞淋巴瘤的经典类型是蕈样肉芽肿和Sézary综合征。近年来,其发病率有逐渐增高的趋势。目前国内外从不同角度对该病的分子生物学进行了较多研究。本文就皮肤T细胞淋巴瘤的肿瘤形成,皮肤浸润,以及预后指标等分子生物学研究进展作了综述。  相似文献   

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阐述皮肤T细胞淋巴瘤向大细胞淋巴瘤转化的临床表现、组织病理、免疫组织化学染色、分子生物学特征、诊断和鉴别诊断与治疗。  相似文献   

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皮肤T细胞淋巴瘤中的MF和Sezary综合征是最先表现在皮肤上的非何杰金病。早期病变局限于皮肤,最好选用局部治疗,例如局部外用氮芥,PUVA及全层皮肤电子束放疗等。对出现红皮病的患者首选体外光置换疗法;全身治疗通常用于病情顽固者及一开始就表现出皮外受累的病人;维甲酸类药物主要用于早期皮肤T细胞淋巴瘤;α-干扰素早期斑块内注射及对晚期病人均有效。  相似文献   

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足部皮肤T细胞淋巴瘤1例   总被引:1,自引:1,他引:0  
报告1例足部皮肤T细胞淋巴瘤。患者男,63岁。左足跟部肿胀4个月,抗感染治疗无效,皮肤组织病理和免疫组化均符合T细胞淋巴瘤。  相似文献   

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T细胞受体基因重排检测皮肤T细胞淋巴瘤   总被引:2,自引:1,他引:1  
为了研究基因诊断在皮肤T细胞淋巴瘤的临床应用,采用聚合酶链反应技术,对60例皮肤淋巴细胞浸润疾患进行了T细胞受体基因重排的检测。结果显示,检出TCR-β和TCR-γ基因克隆重排的有:36/40例CTCL,4/6例可疑蕈样肉芽肿/Sezary综合征和1/1例淋巴瘤样丘疹病。  相似文献   

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Southern印迹分析(SBA)和取和合酶链反应(PCR)法检测早期(IA)和组织学上未确诊的蕈样肉芽肿(MF)皮肤损害的T细胞受体基因重排(TCRGR)阳性率分别为50%以上和19%,有助于诊断。IIA期MF患者特别是伴浅表淋巴结肿大(组织学上大都是反应性增生)的外周血中TCRGR阳性率较高(65%-80%),支持MF早期即为一系统性疾病的理论,但外周血中克隆T细胞和预后相关性则难于确定。Sezary综合征(SS)的TCRGR的发生率在皮肤、淋巴结和外周血中分别为70%、100%和86%。非MF/SS皮肤T细胞淋巴瘤的皮肤 损害和外周血中TCRGR亦较常见。淋巴瘤样丘疹病的皮肤损害和外周血中也可见TCRGR。  相似文献   

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John A.  Zic 《Dermatologic therapy》2009,22(5):407-417
The primary cutaneous T cell lymphomas (CTCL) encompass all malignancies of the T cell where the skin is the primary organ of involvement. The diagnosis of a CTCL variant can be detoured by a number of obstacles including the slow evolution of the disease into a classic clinical and pathologic pattern. A realistic goal of early stage treatment is to reduce the likelihood of progression to a more advanced stage, not to achieve a cure. No studies have adequately compared the different systemic agents in patients with advanced CTCL so the clinician is left to act in the best interest of the patient with what evidence is available. When using the systemic agents, a "start low and go slow" strategy may offer patients several advantages. Dermatologists are uniquely trained to diagnose and to manage all but the most advanced stage patients with CTCL.  相似文献   

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Adult T cell leukemia/lymphoma (ATL) is a malignancy of CD4+ T cells that is endemic in certain areas in Japan. Two types of cutaneous ATL thought to originate from skin include cutaneous tumor and erythematopapule types. Patients with cutaneous ATL show neither leukemic involvement nor invasion of tumor cells into the lymph nodes for at least six months. The differential diagnosis between cutaneous ATL and mycosis fungoides is often difficult. The presence of monoclonal integration of human T lymphotropic virus-I proviral DNA in skin samples is of definitive diagnostic value in cutaneous ATL.  相似文献   

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目的:评价Southern印迹分析(SBA)和聚合酶链反应(PCR)检测原发性皮肤T细胞淋巴瘤(PCTCL)T细胞受体(TCR)基因重排(GR)的意义。方法:以PCR扩增TCRγ的结合Ⅴ(可变区)-J(结合区)序列(TCRγPCR)和SBA分析TCRβ链基因(TCRβSBA)检测克隆性GR。结果:蕈样肉芽肿(MF):TCRγPCR和TCRβSBA检测6例ⅡA期和7例ⅡB期皮损标本的GR分别为5例和4例以及6例和5例,外周血分别有4例、2例和5例、3例示GR;而7例ⅠA期和10例ⅠB期的TCRγGR和TCRβGR皮肤组织为4例、1例和7例、1例,外周血为3例、阴性和4例、1例。1例MFⅡA表现为皮病性淋巴结病患者的淋巴结中证实有GR。疑诊MF:11例患者的皮损和外周血标本经TCRγPCR检测5例皮肤和3例外周血见GR。非蕈样肉芽肿、Sézary综合征的PCTCL:PCR和SBA显示TCRGR分别为皮肤组织占9例/10例和6例/8例,外周血占9例/10例和6例/11例。Sézary综合征和淋巴瘤样丘疹病:2例Sézary综合征外周血和其中1例皮肤标本同时见TCRγGR和TCRβGR;2例淋巴瘤样丘疹病的皮肤标本  相似文献   

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【摘要】 目的 探讨表观遗传抑制因子PcG家族成员果蝇zeste基因增强子的人类同源物1/2(EZH1/EZH2)、胚胎外胚层发育蛋白(EED)及胚胎干细胞抑制蛋白(SUZ12)在常见皮肤T细胞淋巴瘤及淋巴组织增殖性疾病(CTCL/ LPD)中的表达。方法 收集2012—2019年于中国医学科学院皮肤病医院确诊的93例CTCL/LPD及8例扁平苔藓皮损石蜡标本,行免疫组化染色,观察EZH2、EED、SUZ12及EZH1蛋白表达。采用SPSS 25.0软件进行卡方检验及Spearman相关分析。结果 93例中包括44例蕈样肉芽肿(MF)、17例NK/T细胞淋巴瘤(NK/TCL)及原发性皮肤间变大细胞淋巴瘤(PC?ALCL)、淋巴瘤样丘疹病(LyP)、种痘水疱病样淋巴组织增殖性疾病(HV?like LPD)及皮下脂膜炎样T细胞淋巴瘤(SPTCL) 各8例。93例CTCL/LPD中83例(89.2%)EZH2、81例(87.1%)EED、78例(83.9%)SUZ12、37例(39.8%)EZH1阳性;8例扁平苔藓中1例EZH2、8例EZH1阳性,EED、SUZ12全阴性。CTCL/LPD与扁平苔藓4种蛋白的表达分级差异均有统计学意义(χ2分别为41.75、39.74、39.36及32.83,均P < 0.001),且MF、NK/TCL、PC?ALCL、LyP、HV?like LPD及SPTCL与扁平苔藓的表达差异亦均有统计学意义(α = 0.008 3,均P < 0.001)。同时,EZH2与EZH1的表达评分在MF、NK/TCL、PC?ALCL、LyP、HV?like LPD及SPTCL中均呈负相关(rs分别为-0.60、-0.68、-0.89、-0.74、-0.93、-0.80,均P < 0.05)。结论 PcG家族成员EZH2、EED、SUZ12及EZH1在CTCL/LPD中表达异常。  相似文献   

16.
We report a case of an 88‐year‐old woman with a decalvant, erythematous, ulcerated tumor extending from the right temporal to occipital region. Histopathological analysis revealed a dense infiltration of medium‐to‐large‐sized atypical cells throughout the entire dermis. The result of immunohistochemical analysis showed that the infiltrating T cells expressed programmed death‐1 (PD‐1), Bcl‐6 and CXCL13. Flow cytometry analysis showed that CD4+ PD‐1hi T cells also expressed CD10, inducible T‐cell co‐stimulator and CXCR5. On the basis of the clinical appearance and the histopathological findings, we diagnosed the patient with primary cutaneous peripheral T‐cell lymphoma, not otherwise specified. Recently, the concept of primary cutaneous follicular helper T (TFH)‐cell lymphoma was proposed, and in this case, tumor cells clearly expressed TFH‐cell markers. Therefore, we considered this case to be a variant of the entity. Although this entity is still provisional, this case supports the new concept.  相似文献   

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In 2008, a revised World Health Organization (WHO) system of hematological neoplasm classification was promulgated. Between January 1995 and December 2008, 133 new patients with cutaneous lymphomas were seen at the dermatology clinic of Okayama University Hospital. All patients were re-classified according to the revised WHO system. The incidence rates were analyzed and the survival was estimated. Of 133 patients, 106 (79.7%) had primary cutaneous lymphomas (PCLs) and 27 (20.3%) were skin invasion from extracutaneous origin of systemic lymphoma. Compared with several reports from western countries, "mature T-cell and NK-cell neoplasms" was frequent in this study (87% vs. 77 or 72%) because of the occurrence of adult T-cell leukemia/lymphoma (ATLL) and "extranodal NK/T cell lymphoma, nasal type", with less frequent occurrence of "mature B-cell neoplasms" (13% vs. 23 or 28%). Estimated survival of patients with mycosis fungoides was favorable (5-year survival rate 90.6%), but that of the patients with primary cutaneous anaplastic large cell lymphoma (C-ALCL) was extremely less favorable than previously reported (5-year survival rate of 47.4%).  相似文献   

18.
BCL2 and JUNB abnormalities in primary cutaneous lymphomas   总被引:4,自引:0,他引:4  
BACKGROUND: BCL2 is upregulated in nodal and extranodal B-cell non-Hodgkin's lymphomas, with a consequent antiapoptotic effect. However, loss of BCL2 has also been noted in some malignancies, suggesting a different molecular pathogenesis. OBJECTIVES: To investigate genomic and protein expression status of BCL2 and to compare the results with that of JUNB in primary cutaneous lymphomas (PCLs). METHODS: We analysed gene copy number of BCL2 and JUNB in 88 DNA samples from 80 patients with PCL consisting of Sézary syndrome/mycosis fungoides (SS/MF), primary cutaneous B-cell lymphoma (PCBCL) and primary cutaneous CD30+ anaplastic large cell lymphoma (C-ALCL) by the use of real-time polymerase chain reaction (PCR) and immunohistochemistry (IHC). Real-time PCR and IHC findings were subsequently compared with the results of additional fluorescent in situ hybridization (FISH) analysis of 23 cases of SS and Affymetrix cDNA expression microarray study of two primary cutaneous T-cell lymphoma (CTCL) cell lines. RESULTS: Real-time PCR analysis showed loss of BCL2 gene copy number in 22 of 80 PCL cases (28%), including 17 of 42 SS/MF, three of 13 C-ALCL and two of 33 PCBCL samples, and gain of BCL2 in four PCBCL samples. Gain of JUNB was identified in 18 of 71 PCL cases (25%), including nine of 35 SS/MF, seven of 13 C-ALCL and two of 31 PCBCL samples. IHC analysis revealed absent nuclear expression of BCL2 protein in 47 of 73 PCL cases, comprising 28 of 36 SS/MF, eight of eight C-ALCL and 11 of 29 PCBCL cases. In contrast, BCL2 protein expression was detected in 26 of 73 PCL cases, consisting of 18 of 29 PCBCL and eight of 36 SS/MF cases. JUNB protein expression was present in tumour cells from 30 of 33 of SS/MF and eight of eight C-ALCL, and was absent in tumour cells from 18 of 27 PCBCL cases. A comparison between BCL2 and JUNB revealed loss of BCL2 and gain of JUNB in five of 35 SS/MF samples, and expression of JUNB protein and absent BCL2 expression in 25 SS/MF and eight of eight C-ALCL cases. In contrast, expression of BCL2 and absent JUNB expression were detected in 67% of PCBCL cases. Additional FISH analysis revealed deletion of BCL2 in 19 of 23 SS cases (83%), including eight cases with BCL2 loss shown by real-time PCR. Furthermore, Affymetrix expression microarray demonstrated decreased expression of proapoptotic and antiapoptotic genes involved in BCL2 signalling pathways such as BOK, BIM, HRK, RASA1 and STAT2 in two CTCL cell lines with BCL2 loss and absent BCL2 expression. Increased expression of JUNB was also identified in the MF cell line. CONCLUSIONS: These findings provide a comprehensive assessment of BCL2 and JUNB status in PCL, and suggest that there is a selection pressure in a subset of CTCL cases for tumour cells showing BCL2 loss and upregulation of JUNB primarily through chromosomal deletion and amplification, respectively.  相似文献   

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