CAJ | 학술논문

目的探讨Fms样酪氨酸激酶3内部串联重复(FLT3-ITD)的急性髓系白血病(AML)患者的临床特点及预后.方法回顾性分析2011年6月至2014年3月收治的39例FLT3-ITD的AML患者临床资料.结果 FLT3-ITD突变的发生率为11.6％(39/337),39例FLT3-ITD AML患者中,M5所占比例最高(43.6％,17/39),其次为M2(28.2％,11/39);外周血白细胞计数平均为88.63×109/L,其中12例(30.8％)大于100×109/L;骨髓原始细胞比例平均为82.53％,其中33例(84.6％)大于50.00％.13例发生髓外浸润;17例核型正常,9例核型异常,3例未见核分裂象;13例患者伴随NPM1突变,2例伴有KIT突变,3例伴有DNMT3突变.37例接受治疗的患者中,第1个疗程获完全缓解(CR)16例(43.2％),第2个疗程获CR 4例(10.8％),2个疗程累积CR率为54.0％,诱导治疗相关病死率为21.6％ (8/37),死亡原因主要为感染及出血.中位随访时间12个月,20例CR患者的复发率为50.0％(10/20),无复发生存(RFS)率为45.0％(9/20);移植组17例患者的总生存及RFS均优于单纯化疗组的20例患者(P=0.004、0.020).结论 FLT3-ITD AML具有初发时外周血白细胞计数及骨髓原始细胞比例高、与M5亚型高度伴随的临床特点,异基因造血干细胞移植能明显提高患者总生存期及无复发生存期.
목적 탐토Fms양락안산격매3내부천련중복(FLT3-ITD)적급성수계백혈병(AML)환자적림상특점급예후.방법 회고성분석2011년6월지2014년3월수치적39례FLT3-ITD적AML환자림상자료.결과 FLT3-ITD돌변적발생솔위11.6％(39/337),39례FLT3-ITD AML환자중,M5소점비례최고(43.6％,17/39),기차위M2(28.2％,11/39);외주혈백세포계수평균위88.63×109/L,기중12례(30.8％)대우100×109/L;골수원시세포비례평균위82.53％,기중33례(84.6％)대우50.00％.13례발생수외침윤;17례핵형정상,9례핵형이상,3례미견핵분렬상;13례환자반수NPM1돌변,2례반유KIT돌변,3례반유DNMT3돌변.37례접수치료적환자중,제1개료정획완전완해(CR)16례(43.2％),제2개료정획CR 4례(10.8％),2개료정루적CR솔위54.0％,유도치료상관병사솔위21.6％ (8/37),사망원인주요위감염급출혈.중위수방시간12개월,20례CR환자적복발솔위50.0％(10/20),무복발생존(RFS)솔위45.0％(9/20);이식조17례환자적총생존급RFS균우우단순화료조적20례환자(P=0.004、0.020).결론 FLT3-ITD AML구유초발시외주혈백세포계수급골수원시세포비례고、여M5아형고도반수적림상특점,이기인조혈간세포이식능명현제고환자총생존기급무복발생존기.
Objective To analyze the clinical features and prognosis of acute myeloid leukemia (AML) patients with FLT3-ITD mutation.Methods Clinical features and therapeutic effect were retrospectively analyzed in 39 AML patients with FLT3-ITD mutation from June 2011 to March 2014.Results AML patients with FLT3-ITD mutations were identified in 11.6 ％ patients (39/337).The mutation was most frequently found in patients with M5 subtype (43.6 ％,17/39),secondly in patients with M2 type (28.2 ％,11/39).The mean of the peripheral WBC was 88.63×109/L,and there were 30.8 ％ (12/39) patients with WBC ＞ 100×109/L.Mean of blast cell in bone marrow (BM) was 82.53 ％,and there were 84.6 ％ (33/39) patients with more over 50 ％.In addition,among 37 patients,there were 13 patients with extramedullary infiltration,17 patients with normal karyotype,9 patients with abnormal karyotype,13 patients with NPM1 gene mutation,2 patients with KIT gene mutation,3 patients with DNMT3 gene mutation.After treatment,43.2 ％ (16/37) patients achieved complete remission (CR) after first induced therapy,and 10.8 ％ (4/37) patients achieved CR after second induced therapy.The death rate related to induced therapy was 21.6 ％ (8/37).The median follow-up period was 12 months.Relapse rate of the 20 patients after CR was 50.0 ％ (10/20),relapse free survival (RFS) was 45.0 ％ (9/20),and overall survival (OS) and RFS of transplantation group were higher than those of chemotherapy group (P =0.004,0.020).Conclusion AML patients with FLT3-ITD mutation have the characteristics of higher peripheral WBC,higher blast cells in BM and accompanying with M5 subtype.Allo-HSCT can significantly improve OS and RFS in these patients.