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首页> 外文期刊>British Journal of Dermatology >Prognostic factors, prognostic indices and staging in mycosis fungoides and Sézary syndrome: Where are we now?
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Prognostic factors, prognostic indices and staging in mycosis fungoides and Sézary syndrome: Where are we now?

机译：蕈样肉芽肿和塞氏病综合征的预后因素，预后指标和分期：我们现在在哪里？

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Mycosis fungoides is the most prevalent form of primary cutaneous T-cell lymphoma. Patients frequently present with early-stage disease typically associated with a favourable prognosis and survival of 10-35 years, but over 25% may progress to advanced disease with a median survival < 4 years, and just 13 months in those with nodal involvement. Sézary syndrome presents in advanced disease with erythroderma, blood involvement and lymphadenopathy. The Bunn and Lamberg staging system (1979) includes stages IA-IIA (early-stage disease) and IIB-IVB (advanced-stage disease) and provides prognostic information, but some patients with tumour-stage disease (IIB) have a worse prognosis than those with erythrodermic-stage (III). Conversely, patients with plaque-stage (IB) folliculotropic mycosis fungoides may have a worse outcome than those with tumour-stage (IIB). The more recent staging system of the European Organisation for the Research and Treatment of Cancer/International Society for Cutaneous Lymphoma has been designed to reflect tumour burden at different sites. However, this staging system has not been validated prospectively for prognosis. Furthermore, this staging system does not include a detailed measurement of skin tumour burden, as indicated by the modified skin weighted severity assessment tool. This assessment measures body surface area of disease and is weighted to record patch, plaque and tumour to produce a numerical value from 0·5 to 400 and is an established endpoint for clinical studies. Nor does this staging include clinicopathological features associated with a poor prognosis such as folliculotropism. Here we review the clinical, haematological, pathological and genotypic parameters outside the staging system, which may affect survival in mycosis fungoides and Sézary syndrome. Most studies are retrospective and single centre. The identification of poor prognostic factors may be used to develop a prognostic index to use alongside staging, which may be of benefit in mycosis fungoides/Sézary syndrome to identify patients with a potentially poor prognosis. What's already known about topic? The European Organization for the Research and Treatment of Cancer/International Society for Cutaneous Lymphoma published a revised staging system for mycosis fungoides/Sézary syndrome (MF/SS) in 2007 to include tumour, node, metastasis and blood involvement at diagnosis. Survival in MF/SS is diverse and ranges from months to decades. Factors outside staging affect survival. What does this study add? This is a review of clinical, haematological, pathological and genotypic changes affecting survival in MF/SS. The development of an international prognostic index to be adopted alongside staging may aid the management of patients.
机译：蕈样真菌病是原发性皮肤T细胞淋巴瘤的最普遍形式。经常出现早期疾病的患者通常预后良好，生存期为10-35年，但超过25％的患者可能进展为中位生存期<4年的晚期疾病，而淋巴结转移患者仅13个月。 Sézary综合征表现为患有红皮病，血液受累和淋巴结病的晚期疾病。 Bunn和Lamberg分期系统（1979年）包括IA-IIA期（早期疾病）和IIB-IVB期（晚期疾病），并提供了预后信息，但是一些肿瘤期疾病（IIB）的患者预后较差比那些有红皮病阶段的人要多。相反，斑块期（IB）的促毛囊性真菌病真菌病患者的结局可能比肿瘤期（IIB）的患者差。欧洲癌症研究和治疗组织/国际皮肤淋巴瘤学会的最新分期系统旨在反映不同部位的肿瘤负荷。但是，该分期系统尚未经过前瞻性的预后验证。此外，该分期系统不包括皮肤肿瘤负担的详细测量结果，如改良的皮肤加权严重度评估工具所示。该评估测量疾病的体表面积，并加权以记录斑块，斑块和肿瘤，以产生0·5至400的数值，这是临床研究的既定终点。此分期也不包括与不良预后相关的临床病理特征，例如毛囊疏松。在这里，我们回顾了分期系统以外的临床，血液学，病理学和基因型参数，这些参数可能会影响蕈样肉芽肿和塞氏病综合征的生存。大多数研究都是回顾性和单一中心的。不良预后因素的鉴定可用于制定预后指标，并与分期一起使用，这可能对真菌病/塞氏病综合征有益，以鉴定潜在不良预后的患者。关于主题的已知信息？欧洲癌症研究和治疗组织/国际皮肤淋巴瘤协会于2007年发布了修订版的真菌病/塞氏病综合征（MF / SS）分期系统，其中包括肿瘤，淋巴结，转移和诊断时的血液侵袭。 MF / SS的生存时间多种多样，从几个月到几十年不等。分期以外的因素影响生存。这项研究增加了什么？这是对影响MF / SS生存的临床，血液学，病理学和基因型变化的综述。与分期一起采用的国际预后指标的开发可能有助于患者的管理。

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