Whittaker Sean
St Johns Institute of Dermatology, Guys and St Thomas' Hospital, London, UK.
Semin Oncol. 2006 Feb;33(1 Suppl 3):S3-6. doi: 10.1053/j.seminoncol.2005.12.015.
Mycosis fungoides and Sezary syndrome, collectively known as cutaneous T-cell lymphomas (CTCLs), are low-grade, indolent, clonal, non-Hodgkin's lymphomas consisting of CD4+ CD45RO+ T cells with a CLA+ CCR4+ skin-homing phenotype. There are several variants of primary CTCLs with differences in clinical behavior and prognosis. Currently, the precise etiologies of mycosis fungoides and Sezary syndrome are unknown. This article reviews our current understanding of the pathogenetic abnormalities involving genomic mutations, abnormal cDNA expression, and dysregulation of signaling pathways in CTCL.
蕈样肉芽肿和塞扎里综合征统称为皮肤T细胞淋巴瘤(CTCL),是一种低度、惰性、克隆性非霍奇金淋巴瘤,由具有CLA+ CCR4+皮肤归巢表型的CD4+ CD45RO+ T细胞组成。原发性CTCL有几种变体,其临床行为和预后有所不同。目前,蕈样肉芽肿和塞扎里综合征的确切病因尚不清楚。本文综述了我们目前对CTCL中涉及基因组突变、异常cDNA表达和信号通路失调的致病异常的理解。