Department of Dermatology
Department of Pathology, Hospital Universitario Fundación Jiménez Díaz, Madrid.
Haematologica. 2019 Feb;104(2):226-235. doi: 10.3324/haematol.2018.197152. Epub 2019 Jan 10.
Primary cutaneous CD30-positive T-cell lymphoproliferative disorders are the second most common subgroup of cutaneous T-cell lymphomas. They include two clinically different entities with some overlapping features and borderline cases: lymphomatoid papulosis and primary cutaneous anaplastic large cell lymphoma. Molecular studies of primary cutaneous anaplastic large cell lymphoma reveal an increasing level of heterogeneity that is associated with histological and immunophenotypic features of the cases and their response to specific therapies. Here, we review the most significant genetic, epigenetic and molecular alterations described to date in primary cutaneous CD30-positive T-cell lymphoproliferative disorders, and their potential as therapeutic targets.
原发性皮肤 CD30 阳性 T 细胞淋巴增生性疾病是皮肤 T 细胞淋巴瘤的第二大亚组。它们包括两种临床上不同的实体，具有一些重叠的特征和交界性病例：蕈样肉芽肿和原发性皮肤间变性大细胞淋巴瘤。原发性皮肤间变性大细胞淋巴瘤的分子研究显示出越来越大的异质性，这与病例的组织学和免疫表型特征及其对特定治疗的反应相关。在这里，我们回顾了迄今为止在原发性皮肤 CD30 阳性 T 细胞淋巴增生性疾病中描述的最显著的遗传、表观遗传和分子改变，并探讨了它们作为治疗靶点的潜力。
