原发性皮肤 CD4+ 小/中型 T 细胞淋巴组织增生性疾病(SMPLPD):人口统计学、临床、治疗和预后方面的回顾性单中心分析。

IF 3.7 4区 医学 Q1 DERMATOLOGY Clinical and Experimental Dermatology Pub Date : 2024-11-22 DOI:10.1093/ced/llae189
Corrado Zengarini, Alba Guglielmo, Giulia Bandini, Andrea Filippini, Martina Mussi, Claudio Agostinelli, Elena Sabattini, Bianca Maria Piraccini, Alessandro Pileri
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引用次数: 0

摘要

原发性皮肤 CD4+ 小/中 T 细胞淋巴组织增生性疾病(SMPLPD),又称 PCS-TCLPD,是一组主要影响皮肤的罕见血液病。在这项回顾性单中心病例系列研究中,我们旨在调查 SMPLPD 的人口统计学、临床、治疗和预后方面的情况。我们按照世界卫生组织的标准,采用组织病理学和免疫组化方法,收集了 2010 年至今确诊病例的数据。我们共纳入了 22 例患者,中位年龄为 61.50 岁,从临床发病到确诊的中位时间为 3.00 个月。保守边缘手术切除是主要选择,17 例患者的临床症状得到缓解,而非手术治疗,包括放射治疗、高能类固醇治疗和消融激光治疗,有 4 例患者的临床症状得到缓解。临床表现各不相同,但最常见的是上半身单个剧烈结节/丘疹。总之,我们的单中心病例系列为 SMPLPD 提供了宝贵的见解,突出了手术治疗的有效性和非手术治疗的潜力。即使存在争议,SMPLPD 的良性性质也强调了实现肿瘤清除和可接受的美学效果的重要性。
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Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorders: demographic, clinical, therapeutic and prognostic aspects. A retrospective monocentric analysis.

Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorders (PCSM-LPDs), represent a rare group of haematological diseases primarily affecting the skin. In this retrospective single-centre case series study, we aimed to investigate the demographic, clinical, therapeutic and prognostic aspects of PCSM-LPD. We collected data from cases diagnosed between 2010 and the present, employing histopathological and immunohistochemical methods following the World Health Organization criteria. We included 22 patients with a median age of 61.5 years and a median time between clinical onset and diagnosis of 3.0 months. Surgical excision with conservative margins was the primary choice, showing clinical remission in 17 cases, while nonsurgical treatments, including radiotherapy, high-potency steroids and ablative laser, achieved clinical remission in four cases. Clinical presentations varied, but the most common was a single violaceous nodule or papule on the upper body parts. In conclusion, our single-centre case series provides valuable insights into PCSM-LPD, highlighting the effectiveness of surgical treatments and the potential of nonsurgical ones. Even if controversial, the benign nature of PCSM-LPD emphasizes the importance of achieving tumour clearance with acceptable aesthetic outcomes.

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来源期刊
CiteScore
3.20
自引率
2.40%
发文量
389
审稿时长
3-8 weeks
期刊介绍: Clinical and Experimental Dermatology (CED) is a unique provider of relevant and educational material for practising clinicians and dermatological researchers. We support continuing professional development (CPD) of dermatology specialists to advance the understanding, management and treatment of skin disease in order to improve patient outcomes.
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