Kim Y H, Hoppe R T
Department of Dermatology, Stanford University School of Medicine, CA 94305, USA.
Semin Oncol. 1999 Jun;26(3):276-89.
Mycosis fungoides (MF) and the Sézary syndrome are a group of extranodal non-Hodgkin's lymphomas of T-cell origin with primary cutaneous involvement. The group distinguishes itself from other primary cutaneous T-cell lymphomas (CTCLs) by its unique clinical features and histopathology. In its early stages, it often resembles common benign dermatoses, and therefore, a definitive diagnosis can be delayed. The affected T cells are characterized by a predominant CD4+ phenotype with frequent loss of CD7 (pan-T-cell antigen) and often demonstrate T-cell receptor (TCR) rearrangement. The prognosis of patients with MF is highly dependent on the extent and type of skin involvement. The initial cutaneous presentation of MF can be patches, plaques, tumors, or erythroderma. Patients who present with limited patch/plaque disease have an outstanding prognosis with an overall long-term survival that is similar to the expected survival of a matched control population. It is exceedingly rare for patients who present with limited or generalized patch/plaque disease without peripheral lymphadenopathy to have extracutaneous involvement. Therefore, the staging evaluation differs for patients with MF versus patients with other non-Hodgkin's lymphomas and should be tailored to the clinical presentation. Patients who have tumorous or erythrodermic skin involvement have a less favorable prognosis, and patients who present with extracutaneous disease have a poor prognosis. There are multiple therapeutic options for patients with MF and the Sézary syndrome. Selection of a specific treatment plan is based primarily on the clinical stage of the disease. The primary therapy for patients with patch/plaque disease without extracutaneous involvement is a topical regimen, whereas chemotherapy or other aggressive systemic regimens are reserved for those with recalcitrant disease or extracutaneous involvement. There is no evidence that early aggressive systemic therapy is preferable to conservative therapy in the management of limited disease. There are newer combination topical and/or systemic regimens that result in an improved clinical response and possibly a prolonged response duration. For advanced disease, standard therapies are often palliative and successful clinical response is often very short-lived. Therefore, all patients with recalcitrant or extracutaneous disease should be considered for newer investigative therapies.
蕈样肉芽肿（MF）和 Sézary 综合征是一组起源于 T 细胞的结外非霍奇金淋巴瘤，主要累及皮肤。该组疾病凭借其独特的临床特征和组织病理学表现与其他原发性皮肤 T 细胞淋巴瘤（CTCL）相区分。在疾病早期，它常类似常见的良性皮肤病，因此确诊可能会延迟。受累的 T 细胞以 CD4+表型为主，常伴有 CD7（全 T 细胞抗原）缺失，且常显示 T 细胞受体（TCR）重排。MF 患者的预后高度依赖于皮肤受累的范围和类型。MF 的初始皮肤表现可为斑片、斑块、肿瘤或红皮病。表现为局限性斑片/斑块病的患者预后良好，总体长期生存率与匹配的对照人群预期生存率相似。对于表现为局限性或广泛性斑片/斑块病且无外周淋巴结肿大的患者，出现皮肤外受累极为罕见。因此，MF 患者与其他非霍奇金淋巴瘤患者的分期评估不同，应根据临床表现进行调整。有肿瘤性或红皮病性皮肤受累的患者预后较差，出现皮肤外疾病的患者预后不良。MF 和 Sézary 综合征患者有多种治疗选择。具体治疗方案的选择主要基于疾病的临床分期。对于无皮肤外受累的斑片/斑块病患者，主要治疗方法是局部治疗方案，而化疗或其他积极的全身治疗方案则用于治疗难治性疾病或有皮肤外受累的患者。没有证据表明在局限性疾病的治疗中，早期积极的全身治疗优于保守治疗。有更新的局部和/或全身联合治疗方案，可改善临床反应并可能延长反应持续时间。对于晚期疾病，标准治疗通常是姑息性的，成功的临床反应往往非常短暂。因此，所有难治性或有皮肤外疾病的患者都应考虑采用更新的研究性治疗方法。
