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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。
Objective To summarize and analyze the clinical manifestations of S 啨 zary syndrome and to improve the diagnosis and treatment of the disease. Methods One patient with Szaryne syndrome was reported and reviewed. Results The patient was 59 years old and had systemic flushing and pruritus as the main features. The peripheral blood lymphocytes reached 171.9 × 109 / L and the abnormal lymphocytes in the bone marrow reached 33.5%. The patients were relieved by high dose glucocorticoid treatment. Since 1994, a total of 11 cases of S 啨 zary syndrome were reported in our country. 10 males accounted for 90.9% of the total, with an average age of 56.9 ± 19.1 years. Erythroderma and itchy skin were significant skin manifestations. Conclusion S 啨 zary syndrome is a rare T-cell lymphoma, more common in older men, with significant early skin lesions but not typical, easily misdiagnosed as neurodermatitis, eczema and so on.