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167例急性红白血病患者的临床特征与预后

[Clinical characteristics and prognoses of 167 acute erythroleukemia patients].

作者信息

Cheng Wenxiu, Sun Aining, Chen Suning, Qiu Huiying, Yin Jia, Ping Na'na, Wang Qian, Wu Depei

机构信息

Jiangsu Institute of Hematology, The First Affiliated Hospital of Soochow University, Key Laboratory of Thrombosis and Hemostasis of Ministry of Health, Collaborative Innovation Center of Hematology, Soochow University 215006, Suzhou, China.

出版信息

Zhonghua Xue Ye Xue Za Zhi. 2014 Nov;35(11):970-3. doi: 10.3760/cma.j.issn.0253-2727.2014.11.004.

DOI:10.3760/cma.j.issn.0253-2727.2014.11.004
PMID:25417871
Abstract

OBJECTIVE

To observe the biological characteristic and the prognoses in patients with acute erythroleukemia (AEL).

METHODS

The results of 167 patients with newly diagnosed AEL, from January 2003 and June 2013 in the First Affiliated Hospital of Soochow University, were reviewed by MICM.

RESULTS

Flow cytometry analysis indicated that CD13(96.1%), CD33(95.1%), CD117(87.4%) and CD34 (79.4%) were highly expressed in AEL. 56 of 148 (37.8%) AEL patients had a variety of cytogenetic abnormalities, 27 of 148(18.2%) patients were complex karyotype (abnormalities involving 3 or more chromosomes), the abnormalities of chromosomes 3, 5, 7 and 8 were more frequently involved and the most common one was +8, accounting for 35.7% of all abnormal karyotype, followed by 5q- (17.9%). Mutation analysis showed CEBPA mutation ratio of AEL patients was 44.0% (11/25), that of NPM1 as 15.4% (4/26). Initial induced remission rate of AEL was 56.6% (30/53), compared by 33.3% (4/12) of MDSM6. Survival analysis showed that the overall survival in female was better than that in male (P=0.047). The overall survival time of transplantation group is significantly longer than chemotherapy group (P=0.000). The OS of 13-39 years old group was the best, 40-49 years old group took second place, >50 years old group appeared to be the worst.

CONCLUSION

AEL had its own unique biological features, and allogeneic hematopoietic stem cell transplantation could significantly improve its poor prognosis.

摘要

目的

观察急性红白血病(AEL)患者的生物学特征及预后。

方法

回顾性分析2003年1月至2013年6月苏州大学附属第一医院收治的167例初诊AEL患者的MICM结果。

结果

流式细胞术分析显示,AEL中CD13(96.1%)、CD33(95.1%)、CD117(87.4%)和CD34(79.4%)高表达。148例AEL患者中56例(37.8%)存在多种细胞遗传学异常,148例患者中有27例(18.2%)为复杂核型(涉及3条或更多染色体异常),3、5、7和8号染色体异常较为常见,最常见的是+8,占所有异常核型的35.7%,其次是5q-(17.9%)。突变分析显示,AEL患者CEBPA突变率为44.0%(11/25),NPM1为15.4%(4/26)。AEL初始诱导缓解率为56.6%(30/53),MDSM6为33.3%(4/12)。生存分析显示,女性总生存期优于男性(P = 0.047)。移植组总生存时间显著长于化疗组(P = 0.000)。13 - 39岁组OS最好,40 - 49岁组次之,>50岁组最差。

结论

AEL有其独特的生物学特征,异基因造血干细胞移植可显著改善其不良预后。

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