Department of Hematology, People's Liberation Army the General Hospital of Western Theater Command, Chengdu, Sichuan, China.
Department of Pathology, People's Liberation Army the General Hospital of Western Theater Command, Chengdu, Sichuan, China.
Medicine (Baltimore). 2023 Oct 6;102(40):e35413. doi: 10.1097/MD.0000000000035413.
Subcutaneous panniculitis like T-cell lymphoma (SPTCL) is a rare primary cutaneous lymphoma that belongs to peripheral T cell lymphomas, of which the overall prognosis is poor. Chidamide, a deacetylase inhibitor, has been approved for the treatment of peripheral T cell lymphomas. However, due to the rare occurrence of SPTCL, it is currently unknown whether Chidamide is effective for all SPTCL patients and whether there are molecular markers that can predict its therapeutic effect on SPTCL.
The patient was a sixteen-year-old male and underwent subcutaneous nodule biopsy which showed SPTCL. Next-generation sequencing revealed AT-rich interaction domain 1A (ARID1A) mutation, and positron emission tomography/computed tomography showed scattered subcutaneous fluorodeoxyglucose metabolic lesions throughout the body.
During the first 3 CHOP (cyclophosphamide, doxorubicin, vindesine, and prednisone) treatment, the patient relapsed again after remission, and the successive addition of methotrexate and cyclosporine did not make the patient relapsing again. Then, after adding Chidamide to the last 3 CHOP treatment, the patient was relieved again. The patient underwent autologous hematopoietic stem cell transplantation (auto-HSCT) after completing a total of 8 cycles of chemotherapy, and continued maintenance therapy with Chidamide after auto-HSCT. Currently, the patient has been in continuous remission for 35 months.
This case is the first report of a refractory/recurrent SPTCL with ARID1A mutation treated with Chidamide. The treatment of Chidamide on the basis of CHOP plus auto-HSCT therapy achieved good results, suggesting that ARID1A may act as a molecular marker to predict the therapeutic effect of Chidamide on SPTCL patients, which helps to improve the precision of SPTCL treatment and the overall prognosis of SPTCL patients.
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，属于外周 T 细胞淋巴瘤，总体预后较差。西达本胺是一种去乙酰化酶抑制剂，已被批准用于治疗外周 T 细胞淋巴瘤。然而，由于 SPTCL 罕见，目前尚不清楚西达本胺是否对所有 SPTCL 患者有效，以及是否存在分子标志物可以预测其对 SPTCL 的治疗效果。
患者为 16 岁男性，行皮下结节活检，结果显示 SPTCL。下一代测序显示富含 AT 相互作用结构域 1A（ARID1A）突变，正电子发射断层扫描/计算机断层扫描显示全身散在的皮下氟脱氧葡萄糖代谢病变。
在第 3 次 CHOP（环磷酰胺、多柔比星、长春新碱和泼尼松）治疗期间，患者缓解后再次复发，相继加入甲氨蝶呤和环孢素后未再次复发。然后，在最后 3 次 CHOP 治疗中加入西达本胺后，患者再次缓解。患者完成 8 个周期化疗后接受了自体造血干细胞移植（auto-HSCT），并在 auto-HSCT 后继续维持西达本胺治疗。目前，患者已持续缓解 35 个月。
这是首例 ARID1A 突变的难治性/复发性 SPTCL 患者用西达本胺治疗的报告。在 CHOP 联合 auto-HSCT 治疗的基础上加用西达本胺取得了良好的效果，提示 ARID1A 可能作为分子标志物，预测西达本胺治疗 SPTCL 患者的疗效，有助于提高 SPTCL 治疗的精准性，改善 SPTCL 患者的总体预后。
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