Department of Medical Sciences, Section of Dermatology, University of Turin, Torino, Italy.
Department of Health Sciences, Section of Dermatology, University of Florence, Florence, Italy.
Hematol Oncol. 2024 Jan;42(1):e3215. doi: 10.1002/hon.3215. Epub 2023 Aug 30.
Primary cutaneous B-cell lymphomas (PCBCLs) are lymphoproliferative disorders that appear on the skin without evidence of extracutaneous manifestations at the time of diagnosis. There is a lack of evidence-based guidelines for their clinical management due to the availability of very few large scale studies and controlled clinical trials. Here we present and discuss a series of major unmet clinical needs (UCNs) in the management of PCBCLs by a panel of 16 experts involved in research and clinical practice of PCBCL. The Panel produced recommendations on the appropriateness of the clinical decisions concerning the identified clinical needs and proposed research for improving the knowledge needed to solve them. Recommendations and proposals were achieved by multiple-step formalized procedures to reach a consensus after a comprehensive analysis of the scientific literature. Recommendations and proposals lay in the domain of classification uncertainties of PCBCL, optimization of diagnosis, optimization of prognosis, optimization of staging and critical issues on therapeutic strategies with particular focus on new treatments. These recommendations are intended for use not only by experts but above all by dermatologists and hematologists with limited experience in the field of PCBCLs as well as general practitioners.
原发性皮肤 B 细胞淋巴瘤(PCBCL)是一种淋巴增生性疾病,在诊断时出现在皮肤上,没有证据表明有皮肤外表现。由于缺乏大规模研究和对照临床试验,因此缺乏针对其临床管理的循证指南。在这里,我们由参与 PCBCL 研究和临床实践的 16 名专家组成的小组提出并讨论了 PCBCL 管理方面的一系列重大未满足的临床需求(UCN)。专家组就与确定的临床需求相关的临床决策的适当性提出了建议,并提出了研究建议,以提高解决这些问题所需的知识。建议和提案是通过多步骤的正式程序提出的,在对科学文献进行全面分析后达成共识。建议和提案涉及 PCBCL 的分类不确定性、诊断优化、预后优化、分期优化以及治疗策略方面的关键问题,特别关注新的治疗方法。这些建议不仅供专家使用,而且供在 PCBCL 领域经验有限的皮肤科医生和血液科医生以及全科医生使用。