Servicio de Dermatología, Hospital del Mar, Barcelona, España.
Servicio de Dermatología, Hospital del Mar, Barcelona, España.
Actas Dermosifiliogr (Engl Ed). 2021 Jan;112(1):14-23. doi: 10.1016/j.ad.2020.08.003. Epub 2020 Sep 5.
CD30 primary cutaneous lymphomas comprise a large group of malignant lymphoproliferative disorders that present in the skin without extracutaneous involvement at the time of diagnosis. The incidence of these lymphomas is low, at 7 to 10 cases per 100 000 population. Two types, derived from T cells (70%-85%) or B cells (15%-30%), have been identified. Hematologists and oncologists have increasingly recognized the idiosyncrasy of primary cutaneous lymphomas, as reflected in the updated classification of the World Health Organization. However, there remain nuances or small differences to consider when managing these conditions, obliging dermatologists to continue to strive to fully reconcile the various clinical pictures in future reviews of the classification of lymphoid neoplasms. A diagnosis of a primary cutaneous lymphoma is based on clinical, histopathologic, immunophenotypic, and genotypic criteria, particularly evidence of T- or B-cell lymphoid monoclonality in lesions. Also relevant are complementary tests to rule out extracutaneous involvement.
CD30 原发性皮肤淋巴瘤包括一组很大的恶性淋巴增生性疾病,这些疾病在诊断时表现为皮肤受累,无皮肤外受累。这些淋巴瘤的发病率较低,每 10 万人中有 7 至 10 例。已经确定了两种类型,源自 T 细胞(70%-85%)或 B 细胞(15%-30%)。血液学家和肿瘤学家越来越认识到原发性皮肤淋巴瘤的特殊性,这反映在世界卫生组织的最新分类中。然而,在管理这些疾病时仍然需要考虑细微差别或小差异,这迫使皮肤科医生继续努力在未来对淋巴瘤的分类进行审查时,充分协调各种临床表现。原发性皮肤淋巴瘤的诊断基于临床、组织病理学、免疫表型和基因型标准,特别是病变中 T 或 B 细胞淋巴单克隆性的证据。排除皮肤外受累的补充检查也很相关。