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[MLL-AF10 阳性急性白血病的临床特征与预后]

[Clinical features and prognosis in MLL-AF10 positive acute leukemia].

作者信息

Wang Li'na, Qin Yazhen, Jia Jinsong, Zhao Ting, Wang Jing, Yang Shenmiao, Wen Lei, Lu Jin, Huang Xiaojun

机构信息

Peking University People's Hospital, Beijing 100044 , China.

出版信息

Zhonghua Xue Ye Xue Za Zhi. 2015 Oct;36(10):840-3. doi: 10.3760/cma.j.issn.0253-2727.2015.10.007.

Abstract

OBJECTIVE

To analyze the clinical features and prognosis of acute leukemia patients with the mixed lineage leukemia(MLL)gene rearrangements AF10 positive.

METHODS

6 cases with MLL-AF10 positive were analyzed retrospectively, related literatures were reviewed to clarify MLL-AF10 patients'clinical features and prognosis.

RESULTS

The median age of 6 cases was 19.5 years old, 5 patients with fever onset, the onset white blood cells of 4 patients were less than 10×10⁹/L. 5 cases were as M₅ and 1 case M₄ according to FAB classification, the level of fusion gene(RQ-PCR)was 0.23%-22.60% when diagnosed, 4 cases had concomitant WT1 gene mutation, flow cytometry disclosed myeloid phenotype. Of 5 evaluated patients achieved the first complete remission after conventional chemotherapy, 2 cases of complex karyotype died, one case died of sepsis in induction, another died from failing of transplantation. 4 out of 5 transplant recipients gained long term survival.

CONCLUSION

The MLL-AF10 positive patients were mostly young men, the majority FAB classification was M5 or M4, often onset with fever, low white blood cells and low level of fusion gene, usually associated with WT1 mutation. Conventional chemotherapy produced a high response rate, but easy to relapse, while the complex karyotype had a poor prognosis, allo-HSCT may have the potential to improve the prognosis of MLL-AF10 positive patients.

摘要

目的

分析急性白血病患者混合谱系白血病(MLL)基因重排AF10阳性的临床特征及预后。

方法

回顾性分析6例MLL-AF10阳性患者,复习相关文献以明确MLL-AF10患者的临床特征及预后。

结果

6例患者中位年龄19.5岁,5例发热起病,4例起病时白细胞<10×10⁹/L。FAB分类5例为M₅,1例为M₄,确诊时融合基因(RQ-PCR)水平为0.23% - 22.60%,4例伴有WT1基因突变,流式细胞术显示为髓系表型。5例评估患者经传统化疗后首次获得完全缓解,2例复杂核型患者死亡,1例诱导期死于败血症,另1例死于移植失败。5例移植受者中有4例获得长期生存。

结论

MLL-AF10阳性患者多为年轻男性,多数FAB分类为M5或M4,常以发热、白细胞低及融合基因水平低起病,常伴有WT1突变。传统化疗缓解率高,但易复发,复杂核型预后差,异基因造血干细胞移植可能有改善MLL-AF10阳性患者预后的潜力。

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