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CD8细胞毒性T细胞组成,主要位于皮下组织中。由于SPTCL的稀有性,目前尚无标准治疗方法。化疗,放射治疗,免疫抑制剂,造血干细胞移植(HSCT)已被频繁使用,然而,这些治疗方法的效果仍存在争议.在这份报告中,我们在一名47岁女性中发现了一例不寻常的SPTCL病例,其最初症状为非典型。病人开始服用依托泊苷,长春新碱,环磷酰胺,阿霉素,和泼尼松(EPOCH)化疗一旦诊断。经过两个周期的化疗,临床症状无明显改善.随后,组蛋白去乙酰化酶(HDAC)抑制剂西达胺从第三个周期开始加入化疗。经过4个周期的化疗联合西达本胺治疗后,患者逐渐恢复并达到完全缓解(CR)。其次是西达本胺单药治疗维持。治疗后超过1年,她留在CR。我们的案例说明,第一次,西达本胺可以是引起罕见SPTCL长期缓解的有效药物。
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.