Objective: This study aims to explore the clinical characteristics of T-cell large granular lymphocyte leukemia (T-LGLL) patients with STAT3 mutation status and provide a reference for clinical management of such patients. Methods: The clinical data of T-LGLL patients between 2009 and 2019 in Jiangsu Province Hospital were retrospectively analyzed. Differences in baseline clinical data, treatment responses, and survival outcomes in patients with STAT3 mutations or with no mutations were compared. Results: A total of 80 patients were included, including 66 patients without STAT3 mutation and 14 patients (17.5%) with STAT3 mutation. The frequency of Y640F mutation was the highest (42.9%) . Compared with non STAT3 mutation group, STAT3 mutation group had lower HGB (67.5 g/L vs 82.5 g/L, P=0.018) , lower neutrophil count (0.665×10(9)/L vs 1.465×10(9)/L, P<0.001) , higher LDH (229 U/L vs 198 U/L, P=0.041) , higher ferritin (402.5 g/L vs 236.0 g/L, P=0.029) , higher expression rate of TCR Vβ subfamily (89.2% vs 65.4%, P=0.014) and higher proportion of patients with treatment indications (100% vs 74%, P=0.033) . The complete remission rates of STAT3 mutation group and non mutation group were 38.5% and 32.7%, respectively, with no significant difference (P=0.748) . The overall response rate of first-line immunosuppressive therapy in STAT3 mutation group and non mutation group were 69.2% and 69.4%, respectively, with no significant difference (P=1.000) . The median follow-up time was 63 (2-121) months. There was no significant difference in the overall survival time between the two groups (P=0.170) . Conclusions: T-LGLL patients with STAT3 mutations seems to be correlated with an increased tumor burden and high treatment demand, and had a good response to first-line immunotherapies. The prognostic significance of STAT3 mutation in T-LGLL patients requires further validation.
目的: 探讨携带STAT3基因突变的T细胞大颗粒淋巴细胞白血病(T-LGLL)患者的临床特征,为此类患者的临床管理提供参考。 方法: 回顾性分析2009至2019年就诊于江苏省人民医院的T-LGLL患者的临床资料,比较STAT3突变患者与未突变患者的基线临床数据、治疗反应及生存结局。 结果: 共纳入80例患者,STAT3未突变组66例,STAT3突变组14例(17.5%),其中Y640F突变发生频率最高(42.9%)。STAT3突变组与STAT3未突变组相比,HGB减低(67.5 g/L对82.5 g/L,P=0.018),中性粒细胞计数减少(0.665×10(9)/L对1.465×10(9)/L,P<0.001),乳酸脱氢酶升高(229 U/L对198 U/L,P=0.041),铁蛋白升高(402.5 g/L对236.0 g/L,P=0.029),TCR Vβ亚家族表达率升高(89.2%对65.4%,P=0.014),具备治疗指征患者比例升高(100%对74%,P=0.033)。STAT3突变组与未突变组一线免疫抑制治疗的完全缓解率分别为38.5%和32.7%,差异无统计学意义(P=0.748)。STAT3突变组与未突变组一线免疫抑制治疗的总有效率分别为69.2%和69.4%,差异无统计学意义(P=1.000)。中位随访63(2~121)个月,两组总生存时间(均未达到)的差异无统计学意义(P=0.170)。 结论: STAT3基因突变的T-LGLL患者可能有更高的肿瘤负荷和治疗需求,一线应用免疫抑制剂疗效良好。STAT3基因突变对T-LGLL患者预后的意义尚需进一步验证。.
Keywords: Gene, STAT3; Immunosuppression; Leukemia, large granular lymphocyte; Therapy.