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髓系恶性肿瘤的DEK-CAN分子监测有助于治疗分层。

DEK-CAN molecular monitoring of myeloid malignancies could aid therapeutic stratification.

作者信息

Garçon L, Libura M, Delabesse E, Valensi F, Asnafi V, Berger C, Schmitt C, Leblanc T, Buzyn A, Macintyre E

机构信息

Faculté de Medecine, Université Paris-Descartes, INSERM EMI U210 and AP-HP Hématologie-biologique, Hôpital Necker- Enfants Malades, rue de Sèvres, Paris cedex, France.

出版信息

Leukemia. 2005 Aug;19(8):1338-44. doi: 10.1038/sj.leu.2403835.

Abstract

The t(6;9)(p23;q34) is a recurrent chromosomal abnormality observed in 1% of acute myelogenous leukemia (AML), which generates a fusion transcript between DEK and CAN/NUP214 genes. We used a DEK-CAN real-time quantitative (RQ)-PCR strategy to analyze 79 retrospective and prospective samples from 12 patients. Five patients reached DEK-CAN negativity (sensitivity 10(-5)); all underwent early allogeneic hematopoietic stem cell transplantation (median 5.5 months from diagnosis) with some demonstrating molecular positivity at the time of allograft. All four cases in CCR with adequate follow-up (median 18.5 months, range 13--95) demonstrate persistent molecular negativity, whereas all seven patients with persistent DEK-CAN positivity died at a median of 12 months from diagnosis (range 7--27). We conclude that DEK-CAN molecular monitoring by RQ-PCR in t(6;9) malignancies is a useful tool for individual patient management and that molecular negativity is indispensable for survival, but should not be a prerequisite for allografting in this rare, poor prognosis, subset of AML.

摘要

t(6;9)(p23;q34)是一种在1%的急性髓系白血病(AML)中观察到的复发性染色体异常,它会产生DEK基因与CAN/NUP214基因之间的融合转录本。我们采用DEK-CAN实时定量(RQ)-PCR策略分析了12例患者的79份回顾性和前瞻性样本。5例患者达到DEK-CAN阴性(灵敏度为10^(-5));所有患者均接受了早期异基因造血干细胞移植(自诊断起中位数为5.5个月),部分患者在移植时显示分子阳性。所有4例完全缓解(CCR)且随访充分(中位数为18.5个月,范围为13 - 95个月)的病例均显示持续分子阴性,而所有7例持续DEK-CAN阳性的患者自诊断起中位数12个月时死亡(范围为7 - 27个月)。我们得出结论,通过RQ-PCR对t(6;9)恶性肿瘤进行DEK-CAN分子监测是个体患者管理的有用工具,分子阴性对生存至关重要,但不应成为这种罕见的、预后不良的AML亚组患者进行移植的先决条件。

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