The First Clinical Medical College of Lanzhou University, Lanzhou, China.
Department of Hematology, Lanzhou University Second Hospital, Lanzhou, China.
Int J Immunopathol Pharmacol. 2021 Jan-Dec;35:20587384211009342. doi: 10.1177/20587384211009342.
Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare primary cutaneous lymphoma composed of CD8 cytotoxic T-cell that is primarily localized in the subcutaneous tissue. No standard treatments are currently available for SPTCL due to its rarity. Chemotherapy, radiotherapy, immunosuppressive agents, and hematopoietic stem cell transplantation (HSCT) have been used frequently, however, the effects of these treatment approaches remain controversial. In this report, we present an unusual case of SPTCL in a 47-year-old woman whose initial symptoms were atypical. The patient was started on etoposide, vincristine, cyclophosphamide, doxorubicin, and prednisone (EPOCH) chemotherapy once diagnosed. After two cycles of chemotherapy, her clinical symptoms were not significantly improved. Subsequently, histone deacetylase (HDAC) inhibitor chidamide was added to the chemotherapy from the third cycle. She recovered gradually and achieved complete remission (CR) after four cycles of chemotherapy combined with chidamide, followed by chidamide monotherapy for maintenance. More than 1 year after the therapy, she remained in CR. Our case illustrates, for the first time, chidamide can be an effective agent to induce long-term remission for rare SPTCL.
皮下脂膜炎样 T 细胞淋巴瘤(SPTCL)是一种罕见的原发性皮肤淋巴瘤,由主要局限于皮下组织的 CD8 细胞毒性 T 细胞组成。由于其罕见性,目前尚无针对 SPTCL 的标准治疗方法。化疗、放疗、免疫抑制剂和造血干细胞移植(HSCT)经常被使用,然而,这些治疗方法的效果仍存在争议。在本报告中,我们介绍了一例 47 岁女性 SPTCL 不典型首发的罕见病例。该患者确诊后即开始接受依托泊苷、长春新碱、环磷酰胺、多柔比星和泼尼松(EPOCH)化疗。两个周期的化疗后,其临床症状没有明显改善。随后,在第三个周期的化疗中加入组蛋白去乙酰化酶(HDAC)抑制剂西达本胺。经过四个周期的化疗联合西达本胺治疗后,她逐渐恢复,达到完全缓解(CR),随后进行西达本胺单药维持治疗。治疗后 1 年以上,她仍处于 CR。本病例首次表明,西达本胺可以作为诱导罕见 SPTCL 长期缓解的有效药物。