中国麻风皮肤病杂志 ›› 2022, Vol. 38 ›› Issue (7): 466-468.doi: 10.12144/zgmfskin202207466

• 病例报告 • 上一篇    下一篇

维布妥昔单抗治疗Sézary综合征一例

张珂1,邵世宏2,薛宏伟3,陈官芝1,肖淑欣3   

  1. 1青岛大学附属医院皮肤科，山东青岛，266000;2青岛大学附属医院病理科，山东青岛，266000;3青岛大学附属医院淋巴瘤科，山东青岛，266000
  • 出版日期:2022-07-15 发布日期:2022-05-17

Treatment of Sézary syndrome with brentuximab vedotin: a case report

ZHANG Ke1, SHAO Shihong2, XUE Hongwei3, CHEN Guanzhi1, XIAO Shuxin3   

  1. 1 Department of Dermatology, The Affiliated Hospital of Qingdao University, Qingdao 266000, China;2 Department of Pathology, The Affiliated Hospital of Qingdao University, Qingdao 266000, China; 3 Department of Lymphology, The Affiliated Hospital of Qingdao University, Qingdao 266000, China
  • Online:2022-07-15 Published:2022-05-17

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摘要： 患者，男，41岁。全身弥漫性红斑伴严重瘙痒1年，多发淋巴结肿大3个月。PET/CT符合淋巴瘤；淋巴结病理：非霍奇金T细胞淋巴瘤，伴不规则、扭曲、脑回样核；皮肤病理：非霍奇金T细胞淋巴瘤，呈蕈样肉芽肿改变。结合患者症状、体征、影像学、病理及免疫组化结果，以及外周血T细胞基因重排均为阳性，诊断为Sézary综合征。给予化疗等一线药物治疗效果不佳。更换为含有维布妥昔单抗的治疗方案后患者红斑消退，瘙痒消失，淋巴结减小，达到完全缓解。

关键词: Sézary综合征, 皮肤T细胞淋巴瘤, 维布妥昔单抗

Abstract: A 41-year-old male presented with diffuse erythema with severe pruritus for 1 year and multiple lymph node enlargement for 3 months. PET/CT image was consistent with lymphoma, pathology of lymph node showed non-Hodgkin T cell lymphoma, with irregular, twisted, and cerebriform nuclei. Skin pathology showed non-Hodgkin T cell lymphoma presenting with mycosis fungoides. Sézary syndrome was diagnosed according the above examinations and T cell gene rearrangement in peripheral blood. The effect of chemotherapy and other first-line drugs was not good, then the treatment regimen containing brentuximab vedotin was given, with good effect of erythema subsided, pruritus disappeared, lymph nodes decreased.

Key words: Sézary syndrome, cutaneous T-cell lymphoma, brentuximab vedotin