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伴有中枢神经系统受累的皮下脂膜炎样T细胞淋巴瘤长期缓解:一例报告

Long-term remission of subcutaneous panniculitis-like T-cell lymphoma with central nervous system involvement: A case report.

作者信息

Qiu Yajuan, Zhang Dandan, Zhang Mingzhi

机构信息

Department of Oncology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan 450052, P.R. China.

Department of Pathology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan 450052, P.R. China.

出版信息

Oncol Lett. 2016 Jul;12(1):611-614. doi: 10.3892/ol.2016.4635. Epub 2016 May 26.

Abstract

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is an indolent cutaneous T-cell lymphoma with a favourable prognosis. The reported incidence of central nervous system (CNS) involvement in SPTCL is extremely low. SPTCL with CNS involvement is a fatal disease with no optimal treatment. The present study presents the case of a 27-year-old man who initially presented with erythematous nodules on the left buttock and left inguinal lymph node enlargement. A skin biopsy resulted in a diagnosis of SPTCL. Subsequent to diagnosis, the patient developed CNS involvement and underwent treatment of fotemustine, teniposide and dexamethasone, and complete remission was achieved for 78 months. To the best of our knowledge, this is the first case report of secondary CNS SPTCL with long-term remission. Accumulating evidence shows that this CNS-directed regimen can be effective in SPTCL with CNS involvement and in other CNS lymphomas.

摘要

皮下脂膜炎样T细胞淋巴瘤(SPTCL)是一种预后良好的惰性皮肤T细胞淋巴瘤。据报道,SPTCL累及中枢神经系统(CNS)的发生率极低。伴有CNS受累的SPTCL是一种致命疾病,尚无最佳治疗方法。本研究报告了一例27岁男性患者,最初表现为左臀部红斑结节和左腹股沟淋巴结肿大。皮肤活检诊断为SPTCL。诊断后,患者出现CNS受累,并接受了福莫司汀、替尼泊苷和地塞米松治疗,实现了78个月的完全缓解。据我们所知,这是首例继发CNS的SPTCL长期缓解的病例报告。越来越多的证据表明,这种针对CNS的治疗方案对伴有CNS受累的SPTCL及其他CNS淋巴瘤可能有效。

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