André Florine, Guenova Emmanuella, Blanchard Gabriela
Faculté de biologie et médecine, Université de Lausanne, Département de dermatologie et vénéréologie, Centre hospitalier universitaire vaudois, 1011 Lausanne.
Rev Med Suisse. 2022 Mar 30;18(775):578-589. doi: 10.53738/REVMED.2022.18.775.578.
The two main subtypes of primary cutaneous T-cell lymphomas include the most frequent, mycosis fungoides (MF), and the rare leukemic variant, Sézary syndrome (SS). MF presents as cutaneous patches and can progress to plaques, tumors and erythroderma. SS is characterized by the presence of erythroderma, generalized lymphadenopathy and clonal T cells in the peripheral blood, consistent with a poorer prognosis. Histologically, early CTCL lesions are sometimes indistinguishable from more common inflammatory skin diseases and a clinico-pathological correlation is essential for an accurate diagnosis. Except for allogenic stem-cell transplantation, therapy is generally palliative and aims to improve patient quality of life.
原发性皮肤T细胞淋巴瘤的两种主要亚型包括最常见的蕈样肉芽肿（MF）和罕见的白血病变异型 Sézary 综合征（SS）。MF 表现为皮肤斑块，可进展为斑块、肿瘤和红皮病。SS 的特征是存在红皮病、全身淋巴结肿大和外周血中的克隆性 T 细胞，提示预后较差。组织学上，早期皮肤T细胞淋巴瘤病变有时与更常见的炎症性皮肤病难以区分，临床病理相关性对于准确诊断至关重要。除了异基因干细胞移植外，治疗通常是姑息性的，旨在提高患者的生活质量。
