慢性髓系白血病急髓变临床回顾性分析  

Clinical Retrospective Analysis of Chronic Myeloid Leukemia in Myeloid Blast Crisis

作　　者：余锋 姚子龙[1] 李猛[1] 李艳芬[1] 李艳[1] 李文君[1] 李红华[1] 靖彧[1] YU Feng;YAO Zi-Long;LI Meng;LI Yan-Fen;LI Yan;LI Wen-Jun;LI Hong-Hua;JING Yu(Department of Hematology of Chinese PLA General Hospital,Beijing 100853,China;Department of Hematology,Haikou Municipal people′s Hospital & Central South University Xiangya School of Medicine Affiliated Haikou Hospital,Haikou 570208,Hainan Province,China)

机构地区：[1]解放军总医院血液科,北京100853 [2]中南大学湘雅医学院附属海口医院暨海口市人民医院血液科,海南海口570208

出　　处：《中国实验血液学杂志》2019年第4期995-1000,共6页Journal of Experimental Hematology

摘　　要：目的:单中心回顾性分析慢性髓系白血病急髓变患者的临床表现、实验室检查特征及预后。方法:收集解放军总医院2011年6月至2018年5月间10例慢性髓系白血病急髓变患者临床资料,分析临床特征,实验室检查及长期生存情况。结果:10例慢性髓系白血病急髓变患者中位年龄32.5(23-73)岁;9例有慢性病史,中位慢性期17(4-84)个月。10例患者均有脾脏肿大,B超结果显示中位脾厚5.2(4-7.8)cm,中位脾长14.6(11.4-19.8)cm;慢性髓系白血病急髓变时中位外周血白细胞数41.705(11.9-344.41)×10^9/L,中位血小板数159(13-2326)×10^9/L。10例患者均可检测到Ph+染色体及BCR-ABL1融合基因。染色体检查结果中,5例可检测到附加染色体异常,1例为低二倍体,2例为复杂核型。10例患者中有6例进行了ABL1突变检测,其中2例检测到ABL1T315I突变;基因检查中1例合并P53缺失。中位随访时间10.5(0.2-78)个月。5例联合或不联合酪氨酸激酶抑制剂(tyrosine kinase inhibitor,TKI)TKI的化疗并序贯进行异基因造血干细胞移植,移植前3例达第二个慢性期(CP2),其中2例无进展存活至今(分别为移植后67和69个月),1例死于移植相关疾病(移植后7个月脑出血);2例在移植前为NR,移植后均死于疾病复发进展(分别移植后1和3个月)。5例联合或不联合TKI的化疗未行造血干细胞移植的患者均死于疾病进展,中位活存时间6(0.2-22)个月。结论:慢性髓系白血病急髓变患者行联合TKI化疗诱导达CP2状态,序贯异基因造血干细胞移植可延长患者生存期。Objective:To retrospectively analyze the clinical manifestation,laboratorial test features and prognosis of patients with CML in myeloid blast crisis.Methods:The clinical data of 10 patients with CML in myeloid blast crisis admitted in Chinese PLA General Hospital from June 2011 to May 2018 were collected,and their clinical features, laboratorial data and long-term survival were analyzed.Results:The median age of these 10 cases was 32.5 (23-73) years old.Nine cases had chronic phase history.The median chronic phase was 17(4-84) months.All the 10 cases had splenomegaly;B-ultrasonography showed that the median spleen size was 5.2 (4-7.8) cm in thickness,and 14.6 (11.4- 19.8) in length.When chronic myeloid leukemia was in blast crisis,the median WBC count was 41.705(11.9-344.41)×10^9 /L and the median platelet count was 159 (13-2326)×10^9 /L.The Ph+ chromosome and BCR-ABL1 fusion gene coulld be detected in all the cases.The chromosome karyotyping showed that additional chromosome abnormalities were found in 5 cases.One case was of low diploid,and two cases were with complex karyotype.ABL1 mutation was detected in 6 out of these 10 cases.ABL1 T315I mutation was detected in 2 of them and one was with deletion of combined P53 in genetic tests.The median follow-up time was 10.5(0.2-78) months.There were 5 cases treated sequentially by chemotheraphy with or without TKI and allo-HSCT.Three cases reached CP2 before transplantation.Among them,two cases still survived without progression for 67 months and 69 months after the transplantation respectively.One case died of transplantation-related mortality (suffered from cerebral hemorrhage 7 months after the transplantation).Two cases were NR before the transplantation,and both died of disease relapse or progression at the time points of one or three months after the transplantation.Five cases treated by TKI±chemotheraphy and without HSCT succumbed to disease progression.The median time was 6(0.2-22) months.Conclusion:CML patients in myeloid blast crisis treated by chemothe

关 键 词：慢性髓系白血病急髓变 PH^+ BCR-ABL1 造血干细胞移植 脾脏肿大 

分 类 号：R733.72[医药卫生—肿瘤]