Dong X Y, Li Y L, Wu C Y, Liu Y M, Zhang L, Cheng W, Shang B J, Zhang L, Zhu Z M
Institute of Hematology, Henan Provincial People's Hospital; Henan Key Laboratory of Hematopathology; Henan Key Laboratory of Stem Cell Differentiation and Modification, People's Hospital of Zhengzhou University; People's Hospital of Henan University, Zhengzhou 450003, China.
Zhonghua Xue Ye Xue Za Zhi. 2020 Sep 14;41(9):731-736. doi: 10.3760/cma.j.issn.0253-2727.2020.09.005.
This study aims to investigate the characteristics of gene mutation and clinical prognosis in de novo acute myeloid leukemia (AML) patients with myelofibrosis (MF) . From January 1, 2016, to February 1, 2020, 103 newly diagnosed AML patients in Henan Provincial People's Hospital who simultaneously underwent bone marrow biopsy examination were included. They were divided into the AML-MF group (MF grades 1-3) and the AML without MF group (MF grade 0) , and the clinical features, gene alterations, chemotherapy efficacy, and prognosis were compared between the two groups retrospectively. ①MF was confirmed in 44.7% of AML patients (46/103) , of which 84.8% (39/46) were MF-1 and 15.2% (7/46) were MF-2/3, while MF was not confirmed in 55.3% (57/103) of AML patients. The median of WBC in the AML-MF group was significantly higher than in the AML without MF group [11.205 (0.69-191.82) ×10(9)/L 4.64 (0.18-95.10) ×10(9)/L, =0.024]. More patients in the AML-MF group had nucleated erythrocytes in the peripheral blood (43.5% 24.6% , (2)=4.119, =0.042) . All four AML-M(0) patients were in the AML-MF group, while AML without MF group had a higher proportion of AML-M(2) (=0.014) . ②FLT3-ITD and NPM1 mutations were more frequent in the AML-MF group (=0.021 and 0.039) , while CEBPA mutation was more frequent in the AML without MF group (=0.029) . ③The CR rate in the AML-MF group was significantly lower than in the AML without MF group (69.7% 93.2% ) ((2) =7.412, =0.006) . Multivariate analysis showed that MF, especially the grade of fibrosis, was an independent risk factor for CR in de novo AML. ④The 3-year OS of patients in the AML-MF group was significantly lower than in the AML without MF group (20.5% 72.2% , (2)=4.032, =0.045) . Subgroup analysis showed that OS and PFS of AML-MF1 and AML-MF 2/3 groups were also significantly worse than those of the AML without MF group (=0.001) and MF, especially MF ≥2, was an independent marker for inferior OS and PFS in de novo AML (=0.021 and 0.044) . AML-MF has unique laboratory and clinical characteristics. MF is an independent risk factor for CR, OS, and PFS in AML. Evaluation of MF is very significant for therapy efficacy and prognosis judgment in de novo AML.
本研究旨在探讨初诊急性髓系白血病（AML）合并骨髓纤维化（MF）患者的基因突变特征及临床预后。纳入2016年1月1日至2020年2月1日在河南省人民医院同时接受骨髓活检检查的103例新诊断AML患者。将其分为AML-MF组（MF 1-3级）和无MF的AML组（MF 0级），回顾性比较两组的临床特征、基因改变、化疗疗效及预后。①44.7%（46/103）的AML患者确诊为MF，其中84.8%（39/46）为MF-1，15.2%（7/46）为MF-2/3，而55.3%（57/103）的AML患者未确诊MF。AML-MF组白细胞中位数显著高于无MF的AML组[11.205（0.69-191.82）×10⁹/L对4.64（0.18-95.10）×10⁹/L，P =0.024]。AML-MF组外周血有核红细胞患者更多（43.5%对24.6%，χ²=4.119，P =0.042）。所有4例AML-M(0)患者均在AML-MF组，而无MF的AML组AML-M(2)比例更高（P =0.014）。②FLT3-ITD和NPM1突变在AML-MF组更常见（P =0.021和0.039），而CEBPA突变在无MF的AML组更常见（P =0.029）。③AML-MF组完全缓解（CR）率显著低于无MF的AML组（69.7%对93.2%）（χ² =7.412，P =0.006）。多因素分析显示，MF尤其是纤维化程度是初诊AML患者CR的独立危险因素。④AML-MF组患者3年总生存期（OS）显著低于无MF的AML组（20.5%对72.2%，χ²=4.032，P =0.045）。亚组分析显示，AML-MF1组和AML-MF 2/3组的OS和无进展生存期（PFS）也显著差于无MF的AML组（P =0.001），且MF尤其是MF≥2是初诊AML患者OS和PFS较差的独立标志物（P =0.021和0.044）。AML-MF具有独特的实验室和临床特征。MF是AML患者CR、OS和PFS的独立危险因素。评估MF对初诊AML的治疗疗效和预后判断非常重要。
