Cutaneous T-cell lymphoma (CTCL) is a heterogeneous disease group of unknown etiology with a complex immunological background. As CTCL arises from T cells that have a vital role in the antitumor response, their therapy is largely aimed at reversing the immunological mechanisms leading to or manifesting during this malignancy. Early disease stages can be controlled with skin-directed therapy in most CTCL cases. Still, advanced CTCL has a dismal prognosis and warrants systemic therapy. Despite considerable progress in understanding the pathophysiology of the disease and the numerous systemic treatment options available, long-term remission rates with conventional treatments alone are still low. Allogeneic hematopoietic stem cell transplantation is currently the only curative option for advanced CTCL, including mycosis fungoides and Sézary syndrome. The aims of this review is to summarize the recent findings on the immunology of this heterogeneous disease and to present the advances in its clinical management.
Int Arch Allergy Immunol

中文翻译：

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原发性皮肤T细胞淋巴瘤的发病机制和治疗方法：国际变态反应性大肠杆菌（Collegium Internationale Allergologicum（CIA）Update 2020）

皮肤T细胞淋巴瘤（CTCL）是病因不明的异质性疾病，具有复杂的免疫学背景。由于CTCL产生于在抗肿瘤反应中起着至关重要作用的T细胞，因此其治疗主要旨在逆转导致该恶性肿瘤或在此恶性肿瘤中表现出来的免疫机制。在大多数CTCL病例中，可以通过皮肤定向疗法控制疾病的早期阶段。尽管如此，晚期CTCL的预后不良，需要进行全身治疗。尽管在了解该疾病的病理生理学和许多可用的全身治疗选择方面取得了长足的进步，但仅常规治疗的长期缓解率仍然很低。异基因造血干细胞移植目前是晚期CTCL的唯一治疗选择，包括蕈样肉芽肿和塞氏病综合征。
Int Arch过敏免疫