Cutaneous T-cell lymphomas (CTCLs) are a group of lymphoid neoplasms with high relapse rates and no curative treatment other than allogeneic stem cell transplantation (allo-SCT). CTCL is significantly influenced by disruption of JAK/STAT signaling. Therefore, Janus kinase (JAK) inhibitors may be promising for CTCL treatment. This study is a systematic review aiming to investigate the role of JAK inhibitors in the treatment of CTCL, including their efficacy and safety. Out of 438 initially searched articles, we present 13 eligible ones. The overall response rate (ORR) in the treatment with JAK inhibitors in clinical trials was 11–35%, although different subtypes of CTCL showed different ORRs. Mycosis fungoides showed an ORR of 14–45%, while subcutaneous-panniculitis-like T-cell lymphoma (SPTCL) displayed an ORR ranging from 75% to 100%. Five cases were reported having a relapse/incident of CTCL after using JAK inhibitors; of these, three cases were de novo CTCLs in patients under treatment with a JAK inhibitor due to refractory arthritis, and two cases were relapsed disease after graft-versus-host disease treatment following allo-SCT. In conclusion, using JAK inhibitors for CTCL treatment seems promising with acceptable side effects, especially in patients with SPTCL. Some biomarkers, like pS6, showed an association with better responses. Caution should be taken when treating patients with an underlying autoimmune disease and prior immunosuppression.
皮肤T细胞淋巴瘤（CTCL）是一类具有高复发率的淋巴系统恶性肿瘤，除异基因干细胞移植外尚无根治性疗法。JAK/STAT信号通路失调对CTCL发展具有显著影响，因此Janus激酶抑制剂可能成为CTCL治疗的潜在选择。本研究通过系统综述探讨JAK抑制剂治疗CTCL的疗效与安全性。从初步检索的438篇文献中，最终纳入13篇符合标准的研究。临床试验数据显示JAK抑制剂治疗总体缓解率为11%-35%，但不同CTCL亚型存在差异：蕈样肉芽肿缓解率为14%-45%，而皮下脂膜炎样T细胞淋巴瘤缓解率达75%-100%。文献报道5例患者在使用JAK抑制剂后出现CTCL复发或新发病例，其中3例为难治性关节炎患者在接受JAK抑制剂治疗期间新发CTCL，2例为异基因干细胞移植后移植物抗宿主病治疗后复发。研究表明JAK抑制剂治疗CTCL具有良好前景且副作用可控，尤其在皮下脂膜炎样T细胞淋巴瘤患者中疗效显著。部分生物标志物（如pS6）与治疗反应呈正相关。对于存在自身免疫性疾病基础或既往接受免疫抑制治疗的患者需谨慎用药。
肿瘤(癌症)患者之家