皮肤淋巴瘤 第二讲 向表皮性皮肤T细胞淋巴瘤的按分期治疗

向表皮性皮肤T细胞淋巴瘤（epidermotropiccutaneousT－celllymphomas，ECTCL）系恶性辅助T细胞疾病，常开始于皮肤而后累及骨髓、血中淋巴细胞、淋巴结和内脏器官犤１犦，包括具有独特和重叠临床表现的蕈样肉芽肿（Mycosisfungoides，MF）和Sézary综合征（Sézary＇ssyndrome，SS）。近应用聚合酶链反应扩增T－细胞受体γ链基因重排，证明MF在早期即示克隆性T细胞成分系统性侵犯犤２犦，SS患者血中恶性T细胞成分明显扩增犤３犦。ECTCL的病因仍然不明确，病程一般缓慢，在不同发展阶段，生物行为也不一致。多年来已采用很多不同…     

皮肤淋巴瘤 第一讲 向表皮性皮肤T细胞淋巴瘤的分期和预后

向表皮性皮肤T细胞淋巴瘤（epidermotropiccutaneousT－celllymphomas，ECTCL）系恶性辅助T细胞疾病，常开始于皮肤而后累及骨髓、血中淋巴细胞、淋巴结和内脏器官犤１犦，包括具有独特和重叠临床表现的蕈样肉芽肿（mycosisfungoides，MF）和Sézary综合征（Sézary＇ssyndrome，SS）。近应用聚合酶链反应扩增T－细胞受体γ链基因重排，证明MF在早期即示克隆性T细胞成分系统性侵犯犤２犦，SS患者血中恶性T细胞成分明显扩增犤３犦。ECTCL的分期与预后有关。兹阐述ECTCL的分期和预后如下：１分期已提出过用于确定预后和选择治疗…     

第一讲向表皮性皮肤T细胞淋巴瘤的分期和预后

向表皮性皮肤T细胞淋巴瘤(epidermotropic cutaneous T-cell lymphomas,ECTCL)系恶性辅助T细胞疾病,常开始于皮肤而后累及骨髓、血中淋巴细胞、淋巴结和内脏器官[1],包括具有独特和重叠临床表现的蕈样肉芽肿(mycosis fungoides,MF)和Sézary综合征(Sézary's syndrome,SS).近应用聚合酶链反应扩增T-细胞受体γ链基因重排,证明MF在早期即示克隆性T细胞成分系统性侵犯[2],SS患者血中恶性T细胞成分明显扩增[3].ECTCL的分期与预后有关.兹阐述ECTCL的分期和预后如下:     

原发性皮肤T细胞淋巴瘤

原发性皮肤T细胞淋巴瘤属结外非霍奇金淋巴瘤 ,以皮肤内辅助T细胞的单克隆扩增为特征。他与有相同组织学亚型的累及皮肤的原发性淋巴结淋巴瘤在临床及组织学特征、生物学行为及预后都明显不同。如今最常用的原发性皮肤T细胞淋巴瘤分类是EORTC分类 ,HTLV -Ⅰ可能与原发性皮肤T细胞淋巴瘤相关 ,有待进一步验证。原发性皮肤T细胞淋巴瘤有较为独特的免疫表型 ,T细胞受体基因重排检测有助于其早期诊断及疾病的转归和预防的判断     

原发性皮肤T细胞淋巴瘤

原发性皮肤T细胞淋巴瘤属结外非霍奇金淋巴瘤，以皮肤内辅助T细胞的单克隆扩增为特征。他与有相同组织学亚型的累及皮肤的原发性淋巴结淋巴瘤在临床及组织学特征、生物学行为及预后都明显不同。如今最常用的原发性皮肤T细胞淋巴瘤分类是EORTC分类，HTLV-Ⅰ可能与原发性皮肤T细胞淋巴瘤相关，有待进一步验证。原发性皮肤T细胞淋巴瘤有较为独特的免疫表型，T细胞受体基因重排检测有助于其早期诊断及疾病的转归和预防的判断。     

皮肤T细胞淋巴瘤的电镜定量分析

蕈样肉芽肿(MF)与 Sézary 综合征(SS)均属于皮肤 T 细胞淋巴瘤(CTCL),晚期诊断并不困难,但早期往往与许多慢性良性非特异性炎症性皮肤病不易鉴别,长期     

原发性皮肤T细胞淋巴瘤的T细胞受体基因重排分析

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     

皮肤CD8+T细胞淋巴瘤

皮肤CD8+T细胞淋巴瘤罕见,常见于成人.皮肤损害可表现为蕈样肉芽肿(MF)样、Sezary综合征样、全身性银屑病样、播散性湿疹样网状细胞增生病(PR)、限局性PR和皮肤结节等.约半数病例为侵袭性或惰性.瘤细胞常示向表皮性和主要浸润于皮肤附属器周围,示CD8+、CD7+、CD3+,但常丢失CD2和CD5,不常表达活化抗原(CD25、CD30、Iα).此瘤需与富于CD8+T细胞的疾病如皮肤红斑狼疮、银屑病等鉴别,以及与MF之反应性CD8+细胞区别.     

蕈样肉芽肿和Sézary综合征患者中T细胞亚群的异质性

皮肤T细胞淋巴瘤的早期诊断,往往存在着困难。其原因在于皮损活检时组织病理学的变化相当大,也缺乏组织病理学的定量和统一的标准。许多研究表明,用单克隆抗体原位识别T淋巴细胞亚群,有助于提高早期蕈样肉芽肿(MF)诊断的敏感性。其主要依据是对Sézary综合征(SS)病人的循环单个核细胞以及MF、SS病人受累淋巴结的检查。结果表明,循环Sézary细胞以及从受累淋巴结取出的细胞表达辅助性T细胞的表型。然而,在皮肤浸润中T细胞亚群的检测,结果并不一致。有的     

皮肤T细胞淋巴瘤皮损与外周血中TCRγ基因重排的检测

目的：了解已确诊为皮肤T细胞淋巴瘤（CTCL）患者的皮损和外周血中T细胞受体γ链基因重排（TCRγGR）情况。方法：利用PCR测定21例CTCL患者皮损及外周血TCRγGR，扩增产物经琼脂糖及变性梯度凝胶电泳检测。结果：CTCL患者皮损中TCRγGR阳性率显著高于外周血（P〈0．05）；病程≥12个月的患者外周血中TCRγGR阳性率显著高于病程〈12个月的患者（P〈0．05）；年龄≥60岁患者皮损和外周血中TCRγGR阳性率显著高于〈60岁患者（P〈0．05）。结论：在CTCL患者中存在TCRγGR，早期出现在皮损中的TCRγGR可作为CTCL辅助诊断，并有益于CTCL的早期诊断，外周血中检测出TCRγGR可作为皮损阳性结果的补充。     

T-cell receptor gene analysis in cutaneous T-cell lymphomas

An essential property of the immune system is its ability to generate diverse antibody and T-cell mediated responses to virtually any potential foreign particle, The basic molecular mechanisms responsible for producing this extensive diversity have now been elucidated. Each T cell expresses a unique membrane hound T-cell antigen receptor (TCR) which combines with specific antigenic peptides and major histocompatibility complex molecules. The characterization of TCR usage now represents a focal point for many studies of inflammatory and neoplastic disorders. Such studies are helping to clarify the pathogenesis of T-cell mediated diseases and provide the basis for the development of specific therapies. This paper will review several techniques used to identify neoplastic T-cell clones in cutaneous T-cell lymphoma. Similar methods may be used to analyse TCR gene usage in cutaneous inflammatory dermatoses.     

皮肤T细胞淋巴瘤的T细胞受体基因克隆性重排

目的 研究皮肤T细胞淋巴瘤（CTCL）早期诊断。方法 利用PCR方法，设计PCRVγ1-8,Vγ9,Jγ1/γ2特异引物，分析24例各类型CTCL，2例可疑CTCL及4例非特异性红皮病患者的33份示TCRVγ1-8,37份示Vγ9呈MCGR，BCGR或OCGR，尤其是13例早期蕈样肉芽肿，2例可疑CTCL和4例非特异性红皮病均呈TCRγ-GR克隆性扩增带，6例炎性病变标本示TCRγ-GR克隆性，提示他们为“克隆性皮炎”，需长期随访。结论 用PCR发现早期CTCL的PCRγ-GR克隆性，为诊断提供依据。     

Cutaneous T-cell lymphoma

The "Clinical Snapshot" series provides a concise examination of a clinical presentation including history, treatment, patient education, and nursing measures. Using the format here, you are invited to submit your "Clinical Snapshot" to Dermatology Nursing.     

Cutaneous T-cell lymphoma

Cutaneous T-cell lymphoma (CTCL) is a neoplasm of helper T cells whose first manifestations usually appear in the skin. The various forms of CTCL are distinguished by both clinical features and histopathology. Early on, the diagnosis may be difficult to establish because of its numerous, and often non-specific, clinical presentations. Further, the pathological findings of early lesions may lack the diagnostic features observed in well-developed or advanced disease. The diagnosis of CTCL must be considered in any patient with a chronic, therapy-resistant condition of the skin. In patients with non-specific histological findings, a high index of suspicion and multiple biopsies may eventually lead to a diagnosis of CTCL. Once the diagnosis of CTCL is established, accurate staging is essential both for its effect on treatment decisions and for its prognostic value. In general, CTCL is a chronic, slowly progressive disease with a long evolution. The development of tumours is a poor prognostic sign, as is erythroderma. The Sezary syndrome is a distinct form of erythrodermic CTCL that is characterized by exfoliative erythroderma, lymphadenopathy, lymphocytosis, intense pruritus, and circulating large, abnormal lymphocytes (Sezary cells). When death does occur, it is most often due to septicemia. Treatment of CTCL must be tailored to the individual patient. The most commonly employed treatment options are photochemotherapy and topical chemotherapy.     

Subcutaneous T-cell lymphoma

A 70-year-old woman presents with a 2-year history of intermittent, subcutaneous nodules. The patient was otherwise asymptomatic. A biopsy specimen was consistent with a subcutaneous T-cell lymphoma, a rare subset of peripheral T-cell lymphoma; when accompanied by the hemocphagocytic syndrome, it can be rapidly fatal. The histopathologic characteristics and nature of the disease are discussed.     

Panniculitic T-cell lymphoma

Panniculitic T-cell lymphoma is a rare, aggressive variant of cutaneous T-cell lymphoma, with fewer than 100 cases described. The main problem is its diagnosis, as both the clinical and the histological features may simulate benign panniculitis. We present the case of a 34-year-old male patient, who had presented with an indurated plaque, sclerodermiform in appearance, on the front of the right thigh for 4 months, later accompanied by fever and constitutional symptoms. The initial diagnosis was cellulitis, but no clinical improvement was seen despite systemic antibiotic therapy. After two skin biopsies, the patient was diagnosed with panniculitic cutaneous T-cell lymphoma. The patient was treated with 8 cycles of CHOP chemotherapy, with resolution of the symptoms.     

T-cell receptor variable region gene expression in cutaneous T-cell lymphomas

The cutaneus T-cell lymphomas (CTCL) arc a group of diseases characterized by malignant proliferations of CD4 positive T-cells having monoclonally rearranged T-cell receptor (TCR) genes. A recent study using monoclonal antibodies to two TCR β-chain variable (V) region gene products showed preferential expression of the Vβ8 gene product in these tumors. The finding of predominant usage of a single Vβ gene would imply that selection by antigen is important in the etiology of these tumors. We have studied eight cases of cutaneous T-cell lymphoma and one cell line derived from a patient with mycosis fungoides/Sezary syndrome, using an extended panel of antibodies to V region gene products. Contrary to the previous report, in our study expression of the Vβ8 gene product by tumor cells was not observed in any of the cases of CTCL or in the tumor cell line studied; preferential use of any of the variable region genes recognized by the antibodies in the panel was not observed.