Sézary综合征1例报告及文献复习

目的归纳、分析S啨zary综合征的临床表现特征,提高对该病的诊疗水平。方法报告1例S啨zary综合征患者,并复习相关文献。结果患者男59岁,以全身皮肤潮红伴瘙痒为主要特征,外周血淋巴细胞达171.9×109/L,骨髓中异常淋巴细胞达33.5%,经大剂量糖皮质激素等治疗后缓解。1994年至今国内共报道11例S啨zary综合征患者,男性10例占90.9%,平均年龄56.9±19.1岁,以红皮病、剧痒为显著皮肤表现。结论S啨zary综合征是一种较罕见的T细胞淋巴瘤,多见于老年男性,早期皮肤损害显著但不典型,易被误诊为神经性皮炎、湿疹等。

A Case Report and Literature Review of Sézary''''s Syndrome

Objective To improve the diagnostic and therapeutic levels of Sézary’s syndrome by analyzing the cases reported in domestic literatures.Methods A 59-year-old male patient with Sézary’s syndrome was described in detail and the related literatures since 1994 were reviewed.Results This patient experienced a chronic pathogenesis and was characterized with generalized erythroderma and extensive scaling and itching. Lymphocytosis up to 171.9×109/L and high proportion (33.5%) of abnormal lymphocytes in the marrow were present. A remission was obtained after the treatment with high dose of corticosteroid and son on. The total eleven cases were reported since 1994 in China, among which the male patients were 90.9%(10/11) and the mean age was 56.9±19.1 years old.The erythroderma and intensive pruritus were the main skin manifestations.Conclusion Sézary’s syndrome is a rare T-cell lymphoma with obvious cutaneous features. It almost exclusively appears in old males and is often misdiagnosed as generalized neurodermatitis, eczema and so on because of atypical skin manifestations on early stage.