皮肤T细胞淋巴瘤并发原发性皮肤曲霉病1例

报道1例皮肤T细胞淋巴瘤患者并发上曲霉引起的原发性皮肤曲霉病，患者女性，41岁，左胫前2处相连的巨大黑痂，刮取物直接镜检有透明的分枝分隔菌丝，组织病理检查见较多粗细均匀的有隔菌丝，菌丝呈Y型分枝，菌种鉴定为土曲霉。     

原发性皮肤T细胞淋巴瘤1例

原发性皮肤Ｔ细胞淋巴瘤１例康定华患者蒋××，男，５６岁，已婚，干部，常州市人。右足底、脐部、腋部、右上肢、阴囊部相继出现暗红色斑４个月。初于右足底有红色小丘疹、丘疱疹，数目增多后融合成斑片，斑片中央逐渐消退，形成环状，伴有脱屑，自觉瘙痒。半年后，脐部...     

皮肤T细胞淋巴瘤治疗进展

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。     

第四讲 皮肤淋巴瘤皮肤T细胞淋巴瘤向大细胞淋巴瘤转化

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。     

皮肤淋巴瘤 第四讲 皮肤T细胞淋巴瘤向大细胞淋巴瘤转化

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。     

皮肤T细胞淋巴瘤分子生物学研究进展

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。     

皮肤T细胞淋巴瘤的治疗选择

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。     

原发性皮肤T细胞淋巴瘤T细胞受体基因重排检测

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。     

皮肤透明细胞淋巴瘤三例

皮肤透明细胞淋巴瘤（clearcellymphoma）是皮肤T细胞淋巴瘤的一个亚型,比较少见。我们近见３例,报道如下。临床资料：男２例,女１例,年龄３３～５２岁,平均４５．３岁,病程２～５个月,平均３．５个月。２例伴有发热,１例年前有皮肤淋巴瘤病史,３例皮肤损害均表现为红色浸润性斑块,质硬,边界清楚,浸润深浅不一,大小不等,蚕豆大至拳头大,１例压痛明显,１例浸润性斑块中心坏死结痂,１例局限于右乳房（见图１）,２例广泛分布于胸背部及躯干四肢（见图２）,１例伴有口腔粘膜糜烂和溃疡,１例颌下及颈部淋巴结肿大。实验室检…     

T细胞受体基因重排检测皮肤T细胞淋巴瘤

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。     

Erythrodermic syringotropic cutaneous T-cell lymphoma

Syringotropic cutaneous T-cell lymphoma (CTCL) is a rare localized variant of CTCL, characterized histologically by eccrine gland and ductal hyperplasia surrounded by a dense syringotropic lymphocytic infiltrate. Previously reported only in men, we describe the first woman with syringotropic CTCL. Unusually, she presented with erythroderma, cutaneous nodules, poikilodermatous patches, widespread alopecia and lymphadenopathy.     

Phototherapy for cutaneous T-cell lymphoma

Phototherapy has been utilized for decades in the treatment of various dermatologic conditions, including cutaneous T-cell lymphoma (CTCL). Currently, a number of light sources are available, and selection of the specific modality is based on a number of factors, the most important of which is disease stage. The efficacy of broadband ultraviolet B (UVB) is limited to the patch stage, while psoralen and ultraviolet A (PUVA) is capable of clearing plaques and, sometimes, early tumors. Narrowband UVB is also effective for early stages and has practical advantages over PUVA, but more studies are needed to more fully evaluate its role in CTCL. Long-wave ultraviolet A (UVA1) has likewise shown efficacy, supported by findings of apoptosis induction in UVA1-treated cells. Long-term remissions have been reported for PUVA, but in the majority of cases, maintenance therapy was necessary. Although beneficial as monotherapy for early stages of the disease, phototherapy is also a useful adjunct to other modalities such as interferons, retinoids and electron beam therapy. Studies are ongoing to refine protocols for combination therapy, with the goal of improving efficacy, while minimizing adverse effects.     

Ofuji papuloerythroderma evolving to cutaneous T-cell lymphoma

Ofuji papuloerythroderma is an uncommon entity of unknown aetiology, characterized by a pruritic eruption of widespread, red–brown, flat papules that leads to spare skin folds. A number of cases have been described associated with tumour pathology, mainly cutaneous T‐cell lymphomas. We report a new case of Ofuji papuloerythroderma evolving to cutaneous T‐cell lymphoma in an 85‐year‐old woman who had been previously diagnosed with papuloerythroderma 7 years previously.     

Treatment planning in cutaneous T-cell lymphoma

Effective long-term management of cutaneous T-cell lymphoma (CTCL) requires administration of skin-directed therapies such as topically applied nitrogen mustard or photochemotherapy to achieve a complete response in clinically early disease (patch and thin-plaque-phase mycosis fungoides, MF) and often the concomitant administration of well-tolerated drugs with systemic effects such as interferon alfa, bexarotene, methotrexate or extracorporeal photopheresis in more advanced, but not highly aggressive/nontransformed disease (thick plaque or tumor phase MF or erythrodermic CTCL). The author's approach is provided as a guide for dermatologists in private practice.     

Primary cutaneous aggressive epidermotropic CD8(+) cytotoxic T-cell lymphoma with atypical presentation

Primary cutaneous aggressive epidermotropic CD8(+) cytotoxic T-cell lymphoma is characterized by a proliferation of epidermotropic CD8(+) cytotoxic T cells and an aggressive clinical behavior. Patients present with localized or disseminated eruptive papules, nodules and tumors. We report a case of primary cutaneous aggressive epidermotropic CD8(+) cytotoxic T-cell lymphoma with unusual clinical manifestation. The lesion occurred as multiple brownish macules and flat-topped papules on the hands, feet and face in a 25-year-old woman.     

Hydroa vacciniforme-like primary cutaneous CD8-positive T-cell lymphoma

An 8-year-old Taiwanese girl had a 6-month history of a relapsing papulovesicular eruption on her face that resembled hydroa vacciniforme (HV). Histologically, there was a dense infiltration of large atypical lymphocytic cells expressing CD8. TCR-gamma gene rearrangement study revealed a monoclonal band present in the DNA extracted from the specimen. A diagnosis of CD8+ cutaneous T-cell lymphoma (CTCL) was made. The patient was treated with Chinese herbal drugs and her skin lesions waxed and waned. At this writing, 11 months after establishment of the diagnosis, the skin lesions have been limited to the facial area and no definite evidence of systemic involvement is noted. To our knowledge, this is the first case of CD8+ primary CTCL with clinical features resembling HV.     

Interferon in the treatment of cutaneous T-cell lymphoma

Interferons are polypeptides with a broad range of in vivo effects that have shown efficacy in cutaneous T-cell lymphoma (CTCL). Particularly useful is alfa interferon (IFN) which, as a single agent, has shown partial remission rates of > 50% and complete responses of > 20%. Side-effects are predictable, generally well tolerated and dose-related. The efficacy of IFN has increased with combination therapy without any significant increase in attendant side-effects. An update on the specifics of the different IFN subtypes, their inherent biologic activity, pharmacokinetics, efficacy and safety in CTCL is presented in this paper.     

非化疗和化疗治疗皮肤T细胞淋巴瘤的临床疗效比较

目的:比较非化疗与系统性联合化疗治疗皮肤T细胞淋巴瘤(cutaneous T-cell lymphoma,CTCL)的疗效.方法:收集1986年7月-2006年2月在上海瑞金医院住院且分别接受非化疗或系统性联合化疗的32例CTCL患者的临床资料进行回顾性分析,比较两组患者的治疗效果.结果:两组患者的缓解率差异无统计学意义(P＞0.05);化疗组患者获得缓解的时间较短(P＜0.05);非化疗组患者的缓解期(中位数39个月)长于化疗组(中位数3.7个月)(P＜0.01);非化疗组患者的总生存率(52.94%)高于化疗组(8.33%)(P＜0.05);非化疗组患者的总生存期(中位数106.83个月)长于化疗组(中位数10.7个月)(P＜0.01).结论:系统性联合化疗治疗CTCL病情缓解较快;但非化疗方法能获得较长的缓解期、较高的生存率以及较长的总生存期.