Early clinical manifestations of Sezary syndrome: A multicenter retrospective cohort study
Early clinical manifestations of Sezary syndrome: A multicenter retrospective cohort study
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DOI：
10.1016/j.jaad.2017.05.036
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发表时间：
2017-10-01
影响因子：
13.8
通讯作者：
Gniadecki, Robert
中科院分区：
文献类型：
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作者：
Mangold, Aaron R.;Thompson, Agnieszka K.;Gniadecki, Robert
背景：经典型塞扎里综合征（SS）是由红皮病、全身淋巴结病和白血病血液受累定义的。临床观察表明，SS最初是一种非红皮病性疾病。目的：描述SS患者的早期临床特征。方法：对1976- 2015年确诊的263例SS患者进行回顾性、多中心病历分析。结果：红皮病是最早记录的皮肤症状的SS，只有25.5%的情况下，虽然大多数患者（86.3%）最终发展红皮病。在初次就诊时无红皮病的患者中，SS的最初皮肤体征为非特异性皮炎（49%）、特应性皮炎样皮疹（4.9%）或蕈样肉芽肿斑和斑块（10.6%）。平均诊断延迟为4.2年的整体，2.2年的情况下，涉及红皮病在最初的presentation.Limitations，和5.0年的情况下，不涉及红皮病在最初presentation. This研究是回顾性的。对于常规治疗无效的非红皮病性皮炎，应考虑早期SS。在这些情况下，应考虑通过PCR检查血液中的单克隆T细胞受体重排，并通过流式细胞术鉴定扩增或异常的T细胞群。
Background: Classic Sezary syndrome (SS) is defined by erythroderma, generalized lymphadenopathy, and leukemic blood involvement. Clinical observations suggest that SS begins as a nonerythrodermic disease.Objective: To describe the early clinical characteristics of patients with SS.Methods: A retrospective, multicenter chart review was performed for 263 confirmed cases of SS diagnosed during 1976-2015.Results: Erythroderma was the earliest recorded skin sign of SS in only 25.5% of cases, although most patients (86.3%) eventually developed erythroderma. In patients without erythroderma during their initial visit, the first cutaneous signs of SS were nonspecific dermatitis (49%), atopic dermatitis-like eruption (4.9%), or patches and plaques of mycosis fungoides (10.6%). The mean diagnostic delay was 4.2 years overall, 2.2 years for cases involving erythroderma at the initial presentation, and 5.0 years for cases not involving erythroderma at the initial presentation.Limitations: This study is retrospective.Conclusion: Erythroderma is uncommon as an initial sign of SS. Early SS should be considered in cases of nonerythrodermic dermatitis that is refractory to conventional treatments. In these cases, examination of the blood by PCR for monoclonal T-cell receptor rearrangement and by flow cytometry to identify an expanded or aberrant T-cell population should be considered.