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Abstract：
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.
摘要：
皮肤T细胞淋巴瘤（CTCL）是一组具有高复发率且除同种异体干细胞移植（allo-SCT）外没有治愈性治疗的淋巴肿瘤。CTCL受到JAK/STAT信号传导中断的显著影响。因此,Janus激酶（JAK）抑制剂可能有望用于CTCL治疗。本研究是一项系统综述，旨在探讨JAK抑制剂在CTCL治疗中的作用。包括其功效和安全性。在最初搜索的438篇文章中，我们提供13个合格的。临床试验中使用JAK抑制剂治疗的总反应率（ORR）为11-35％，虽然不同亚型的CTCL显示不同的ORR。真菌病显示14-45%的ORR，而皮下脂膜炎样T细胞淋巴瘤（SPTCL）显示的ORR范围为75％至100％。据报道，5例使用JAK抑制剂后CTCL复发/事件；其中，3例因难治性关节炎而接受JAK抑制剂治疗的患者为从头CTCL，2例患者在同种异体SCT后接受移植物抗宿主病治疗后复发。总之,使用JAK抑制剂进行CTCL治疗似乎具有可接受的副作用，特别是SPTCL患者。一些生物标志物，像pS6一样，显示出与更好的反应的关联。治疗患有潜在自身免疫性疾病和先前免疫抑制的患者时应谨慎。
