维布妥昔单抗治疗Sézary综合征一例

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。

Treatment of Sézary syndrome with brentuximab vedotin: a case report

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.