Siegel R S, Pandolfino T, Guitart J, Rosen S, Kuzel T M
Departments of Hematology/Oncology and Dermatology, Robert H. Lurie Comprehensive Cancer Center, Northwestern University Medical School, Chicago, IL, USA.
J Clin Oncol. 2000 Aug;18(15):2908-25. doi: 10.1200/JCO.2000.18.15.2908.
Primary cutaneous T-cell lymphomas (CTCLs) encompass a wide variety of lymphomas that are characterized by the localization of the malignant lymphocytes to the skin at presentation. Advances in molecular biologic techniques, including immunophenotyping and gene rearrangement studies to determine clonality, have led to more frequent diagnosis of CTCL as well as more consistent subclassification of these entities. However, there continues to be confusion in the classification, prognosis, and management of patients with CTCL. The purpose of this review is to present a summary of the diagnosis, prognosis, and treatment of CTCL, with specific emphasis on mycosis fungoides (MF) and Sézary syndrome (SS). We also present a detailed discussion of the entities that make up the differential diagnosis of CTCL.
We reviewed the medical literature on CTCL and other diseases that make up the differential diagnosis of CTCL.
MF and SS are the most common forms of CTCL. The etiology of this disease is still unknown. Patients may go for months to years with skin abnormalities before being diagnosed. MF/SS is an indolent disease and patients with T1 disease have a normal life expectancy. Patients who undergo transformation to large-cell lymphoma (8% to 23% of patients) have a poor prognosis, with mean survival ranging from 2 to 19 months. Treatment for MF/SS continues to be palliative. There are many new therapies that are currently being investigated in clinical trials, and the DAB(389)IL-2 fusion protein was recently approved for the treatment of refractory MF/SS.
原发性皮肤T细胞淋巴瘤（CTCL）涵盖多种淋巴瘤，其特征是恶性淋巴细胞在发病时定位于皮肤。分子生物学技术的进展，包括用于确定克隆性的免疫表型分析和基因重排研究，使得CTCL的诊断更为频繁，这些实体的亚分类也更为一致。然而，CTCL患者的分类、预后和管理仍存在困惑。本综述的目的是总结CTCL的诊断、预后和治疗，特别强调蕈样肉芽肿（MF）和塞扎里综合征（SS）。我们还详细讨论了构成CTCL鉴别诊断的各种实体。
我们回顾了关于CTCL以及构成CTCL鉴别诊断的其他疾病的医学文献。
MF和SS是CTCL最常见的形式。这种疾病的病因仍然不明。患者在被诊断之前可能出现皮肤异常数月至数年。MF/SS是一种惰性疾病，T1期疾病患者的预期寿命正常。转化为大细胞淋巴瘤的患者（占患者的8%至23%）预后较差，平均生存期为2至19个月。MF/SS的治疗仍然是姑息性的。目前有许多新疗法正在临床试验中进行研究，DAB(389)IL-2融合蛋白最近被批准用于治疗难治性MF/SS。
