PI3K抑制剂联合阿扎胞苷治疗复发/难治血管免疫母细胞性T细胞淋巴瘤5例研究

PI3K inhibitor combined with azacitidine for relapsed/refractory angioimmunoblastic T-cell lymphoma:a study of 5 cases

目的探索PI3K抑制剂联合阿扎胞苷治疗复发/难治血管免疫母细胞性T细胞淋巴瘤(r/r AITL)的疗效及安全性。方法回顾性分析2023年3月至2023年11月就诊于苏州大学附属第一医院血液科的5例r/r AITL患者采用PI3K抑制剂联合阿扎胞苷挽救性治疗的疗效及安全性。结果5例患者中,男性3例,女性2例,中位年龄58(50~86)岁。Ann Arbor分期均为Ⅲ~Ⅳ期,其中3例骨髓累及,2例自体造血干细胞移植后复发,3例合并EB病毒感染。4例患者高通量测序(NGS)检测出RHOA、TET2、IDH2、DNMT3A等1个或多个突变。中位随访时间286(153~397)d。5例患者均获得缓解,其中2例完全缓解(CR),3例部分缓解(PR);中位无进展生存时间(PFS)和总生存时间(OS)均未达到。相关不良反应以1~2级为主,没有患者发生严重的致死性不良反应。结论r/r AITL患者缺乏有效的拯救治疗方案,预后差。PI3K抑制剂联合阿扎胞苷方案成功拯救5例r/r AITL,且不良反应少、耐受性好,值得进一步推广应用。

Objective To investigate the efficacy and safety of PI3K inhibitors combined with azacitidine in the treatment of relapsed/refractory angioimmunoblastic T cell lymphoma(r/r AITL).Methods A retrospective analysis of the eficacy and safety of 5 r/r AITL patients treated by PI3K inhibitor combined with azacitidine in the Department of Hematology,the First Affiliated Hospital of Soochow University from March 2023 to November 2023.Results There were 3 male and 2 female among the five patients,with a median age of 58(range 50-86)years at diagnosis.All patients Ann Arbor stages were II-IV,including three with bone marrow involvement,two experiencing relapsepost-autologous hematopoietic stem cell transplantation and three with EB virus infection.One or more gene mutations such as RHOA,IDH2,TET2,DNMT3A and EP300 were detected by Next-Generation Sequencing(NGS)in 4 patients.All five patients achieved remission,with 2 achieving complete response(CR)and 3achieving partial response(PR).The median follow-up was 286(153-397)days,andmedian progression free survival(PFS)and overall survival(OS)werenot reached.Adverse events were primarily grade 1-2,which were effectively controlled after supportive treatment,and no severe fatal adverse reactions occurred.Conclusion The patients with r/r AITL lacked effective rescue treatment options and had a poor prognosis,the PI3K inhibitor combined with azacitidine regimen successfully rescued five cases of r/r AITL with few adverse reactions and good tolerability,which is worthy of further promotion and application.