Marzano Angelo V, Vezzoli Pamela, Fanoni Daniele, Venegoni Luigia, Berti Emilio
Institute of Dermatological Sciences, Istituto di Ricovero e Cura a Carattere Scientifico Fondazione Ospedale Maggiore Policlinico, Mangiagalli e Regina Elena, University of Milan, Milan, Italy.
J Am Acad Dermatol. 2009 Aug;61(2):348-55. doi: 10.1016/j.jaad.2008.11.894.
Regulatory T (Treg) cells, which represent 5% to 10% of peripheral T cells, regulate the activities of T-cell subsets by performing immunosuppressive functions and thus preventing the development of autoimmune responses. The majority of Treg cells are CD4+, CD25+, and FOXP3+. Recently, it has been demonstrated that the tumor cells in adult T-cell leukemia lymphomas can function as Treg, raising the question of whether any variant of primary cutaneous T-cell lymphoma may also express a regulatory phenotype. We describe an extraordinary case of primary cutaneous T-cell lymphoma clinically characterized by protean cutaneous manifestations and histologically showing a pattern consistent with epidermotropic pleomorphic medium-/large-cell primary cutaneous T-cell lymphoma. The majority of neoplastic cells were CD4+ CD25+ T cells and strongly expressed FOXP3. With this background, the current case, characterized by an aggressive course requiring polychemotherapy, may support the existence of lymphoproliferative malignancies of Treg cells.
调节性T（Treg）细胞占外周T细胞的5%至10%，通过发挥免疫抑制功能来调节T细胞亚群的活性，从而防止自身免疫反应的发生。大多数Treg细胞为CD4+、CD25+和FOXP3+。最近的研究表明，成人T细胞白血病淋巴瘤中的肿瘤细胞可发挥Treg细胞的功能，这就引发了一个问题，即原发性皮肤T细胞淋巴瘤的任何变体是否也可能表达调节表型。我们描述了一例原发性皮肤T细胞淋巴瘤的特殊病例，其临床特征为多变的皮肤表现，组织学显示与亲表皮性多形性中/大细胞原发性皮肤T细胞淋巴瘤一致的模式。大多数肿瘤细胞为CD4+ CD25+ T细胞，并强烈表达FOXP3。在此背景下，本例以需要多药化疗的侵袭性病程为特征，可能支持Treg细胞淋巴增殖性恶性肿瘤的存在。
