成人急性巨核细胞白血病13例临床分析  

Clinical analysis of 13 cases of adult acute megakaryoblastic leukemia

作　　者：刘云涛 惠岩 林冬 李艳 宫本法 刘兵城 邱少伟 魏辉 王建祥 LIU Yun-tao;HUI Yan;LIN Dong;LI Yan;GONG Ben-fa;LIU Bing-cheng;QIU Shao-wei;WEI Hui;WANG Jian-xiang(State Key laboratory of Experimental Hematology,National Clinical Research Center for Blood Diseases,Institute of Hematology&Blood Diseases Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College,Tianjin 300020,China)

机构地区：[1]中国医学科学院血液病医院(中国医学科学院血液学研究所)实验血液学国家重点实验室,国家血液系统疾病临床医学研究中心,天津300020

出　　处：《中国实用内科杂志》2022年第1期69-72,共4页Chinese Journal of Practical Internal Medicine

基　　金：国家自然科学基金青年科学基金(81800173)。

摘　　要：目的分析成人急性巨核细胞白血病(acute megakaryoblastic leukemia,AMKL)患者临床资料,以期了解AMKL的诊治现状,提高该病的诊断准确性。方法收集2010年1月至2020年10月在中国医学科学院血液病医院(中国医学科学院血液学研究所)初诊为AMKL的13例成人AMKL患者临床特征及实验室检查结果、治疗及转归情况,并进行文献复习。结果13例患者中位年龄57(15~68)岁,临床症状以乏力、发热、皮肤黏膜出血为主,可见肝脾大。骨髓涂片有5例患者原始细胞比例低于20%,但综合巨核细胞免疫化学染色(CD41阳性)、流式细胞免疫分型分析(CD36阳性、CD41阳性、CD42b阳性或CD61阳性)或病理活组织免疫化学染色结果(CD41阳性或CD61阳性)考虑诊断为AMKL。13例患者中位生存期为2.5(1.0~13.0)个月。结论成人AMKL发病率低、预后差。流式细胞免疫分型结合骨髓细胞形态、巨核酶标及免疫组化有助于AMKL的诊断。Objective To analyze the clinical characteristics and current therapeutic status of adult patients with acutemegakaryoblastic leukemia(AMKL)in order to improve the diagnostic accuracy.Methods From January 2010 to October2020,the clinical characteristics,laboratory examination results,treatment and prognosis of 13 adult patients with AMKLin Institute of Hematology&Blood Diseases Hospital were collected and the literature was reviewed.Results The medianage of 13 patients was 57(15-68)years old,with the main clinical symptoms of fatigue,fever,petechia andhepatosplenomegaly.The proportion of blasts in bone marrow smear was less than 20% in 5 cases;however,the diagnosis ofAMKL was confirmed based on megakaryozyme(CD41 positive),flow cytometry(CD36,CD41,CD42 b or CD61 positive)or immunohistochemistry(CD41 or CD61 positive).The median survival time of 13 patients was 2.5(1.0-13.0)months.Conclusion Adult AMKL is a rare disease and has a poor prognosis.Flow cytometry combined with bone marrowmorphology,megakaryozyme and immunohistochemistry is helpful for the diagnosis of AMKL.

关 键 词：成人 急性巨核细胞白血病 诊断 预后 

分 类 号：R55[医药卫生—血液循环系统疾病]