蕈样肉芽肿伴大细胞转化的诊疗进展

Progress in the diagnosis and treatment of mycosis fungoides with large cell transformation

蕈样肉芽肿是原发于皮肤记忆性辅助T细胞的非霍奇金淋巴瘤,是皮肤T细胞淋巴瘤最为常见的亚型。大细胞转化是原发性皮肤T细胞淋巴瘤的一种特殊形式。其病因及发病机制尚不明确,临床表现及辅助检查无特异性。目前对患者是否需要积极治疗具有争议。本文系统性回顾蕈样肉芽肿伴大细胞转化的病因及发病机制、临床表现、诊断、治疗进展及预后,为临床医生对该病的认识、诊断和治疗提供帮助。

Mycosis fungoides is a non-Hodgkin lymphoma originating from skin-resident memory helper T cells,representing the most common subtype of cutaneous T-cell lymphoma.Large cell transformation is a unique form of primary cutaneous T-cell lymphoma with unclear etiology and pathogenesis.Clinical manifestations and auxiliary examinations lack specificity.Whether patients need aggressive treatment is controversial.This article systematically reviews the etiology,pathogenesis,clinical manifestations,diagnosis,treatment,and prognosis of mycosis fungoides with large cell transformation,offering insights for its diagnosis and management.