Cerroni Lorenzo
Research Unit Dermatopathology, Department of Dermatology, Medical University of Graz, Auenbruggerplatz 8, 8036 Graz, Austria.
Semin Diagn Pathol. 2017 Jan;34(1):3-14. doi: 10.1053/j.semdp.2016.11.001. Epub 2016 Nov 28.
Primary cutaneous lymphomas represent a broad group of diseases with different clinical, histopathological, phenotypic, molecular, and prognostic features. All cutaneous lymphomas share the same tropism of neoplastic lymphocytes for the skin, but precise classification is paramount for proper management of the patients. Primary cutaneous lymphomas are classified according to the schemes proposed by the European Organization for Research and Treatment of Cancer (EORTC)-Cutaneous Lymphomas Task Force together with the World Health Organization (WHO) in 2005, and the WHO classification of 2008 with the 2016 update. The recognition of organ-based lymphomas, including cutaneous lymphomas, reflects a shift in the approach to lymphoproliferative disorders, and represents one of the major advances in the WHO classification of hematological tumors. Future studies should be aimed at shedding light on the many grey areas of cutaneous lymphomas (particularly the diagnosis and nomenclature of early mycosis fungoides and variants), and at gathering more data on the disorders that are still listed as provisional entities in the WHO classification.
原发性皮肤淋巴瘤是一组具有不同临床、组织病理学、表型、分子和预后特征的疾病。所有皮肤淋巴瘤的肿瘤性淋巴细胞均具有相同的皮肤嗜性,但准确分类对于患者的恰当管理至关重要。原发性皮肤淋巴瘤根据欧洲癌症研究与治疗组织(EORTC)皮肤淋巴瘤工作组与世界卫生组织(WHO)于2005年提出的方案,以及2008年WHO分类及其2016年更新版本进行分类。对包括皮肤淋巴瘤在内的基于器官的淋巴瘤的认识反映了淋巴增殖性疾病研究方法的转变,也是WHO血液肿瘤分类的主要进展之一。未来的研究应致力于阐明皮肤淋巴瘤的诸多模糊领域(尤其是早期蕈样肉芽肿及其变异型的诊断和命名),并收集更多关于在WHO分类中仍列为暂定实体的疾病的数据。