Lezama Lhara Sumarriva, Gratzinger Dita
From the Department of Pathology, Mount Sinai St Luke's, Icahn School of Medicine at Mount Sinai, New York, New York (Dr Lezama); and the Department of Pathology, Stanford University School of Medicine, Stanford, California (Dr Gratzinger).
Arch Pathol Lab Med. 2018 Jan;142(1):139-142. doi: 10.5858/arpa.2016-0352-RS.
Primary cutaneous lymphomas are defined as non-Hodgkin lymphomas that present in the skin with no evidence of extracutaneous disease at the time of diagnosis. Mycosis fungoides is the most common type of primary cutaneous T-cell lymphoma, representing almost 50% of primary cutaneous T-cell lymphomas, and primary cutaneous CD30 T-cell lymphoproliferative disorders are the second most common group (30%). Transformed mycosis fungoides is usually CD30 and can involve multiple nodal sites; other primary cutaneous CD30 T-cell lymphoproliferative disorders can also involve draining regional nodes. Nodal involvement by CD30 T-cell lymphoproliferative disorders can mimic classical Hodgkin lymphoma, which can aberrantly express T-cell antigens. The aim of this article is to briefly review salient clinical, histologic, immunophenotypic, and molecular features that can be used to distinguish lymph node involvement by CD30 cutaneous T-cell lymphomas and lymphoproliferative disorders from classical Hodgkin lymphoma, a clinically important differential diagnosis that represents a challenging task for the pathologist.
原发性皮肤淋巴瘤被定义为非霍奇金淋巴瘤，其在诊断时表现为皮肤病变，且无皮肤外疾病的证据。蕈样肉芽肿是原发性皮肤T细胞淋巴瘤最常见的类型，占原发性皮肤T细胞淋巴瘤的近50%，原发性皮肤CD30 T细胞淋巴增殖性疾病是第二常见的类型（30%）。转化型蕈样肉芽肿通常表达CD30，可累及多个淋巴结部位；其他原发性皮肤CD30 T细胞淋巴增殖性疾病也可累及引流区域淋巴结。CD30 T细胞淋巴增殖性疾病累及淋巴结可类似经典霍奇金淋巴瘤，后者可异常表达T细胞抗原。本文旨在简要综述一些显著的临床、组织学、免疫表型和分子特征，这些特征可用于区分CD30皮肤T细胞淋巴瘤和淋巴增殖性疾病累及淋巴结与经典霍奇金淋巴瘤，这是一项重要的临床鉴别诊断，对病理学家来说是一项具有挑战性的任务。
