Long FLT3 internal tandem duplications and reduced PML-RARα expression at diagnosis characterize a high-risk subgroup of acute promyelocytic leukemia patients

Long FLT3 internal tandem duplications and reduced PML-RARα expression at diagnosis characterize a high-risk subgroup of acute promyelocytic leukemia patients
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DOI:
10.3324/haematol.2009.015073
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发表时间:
2010-05-01
期刊:
HAEMATOLOGICA-THE HEMATOLOGY JOURNAL
影响因子:
--
通讯作者:
Gonzalez, Marcos
Gonzalez, Marcos
中科院分区:
其他
文献类型:
--
作者:
Carmen Chillon, Maria;Santamaria, Carlos;Gonzalez, Marcos

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FLT 3基因的内部串联重复FLT 3-ITD突变在急性早幼粒细胞白血病(APL)患者中很常见,但其临床影响仍存在争议。设计和方法我们分析了FLT 3-ITD突变水平和大小以及FLT 3-D835点突变的预后意义,在西班牙LPA 96(n=43)或LPA 99(n=86)PETHEMA试验中入选的129例APL患者中,PML-RAR α表达和其他预测因素。在21%和9%的患者中分别检测到ITD和D835突变。ITD突变型/野生型比例增加或ITD大小较长的患者显示较短的5年无复发生存期(RFS)(P=0.048和P
BackgroundInternal tandem duplications of the FLT3 gene (FLT3-ITDs) are frequent in patients with acute promyelocytic leukemia (APL), however its clinical impact remains controversial.Design and MethodsWe analyzed the prognostic significance of FLT3-ITD mutant level and size, as well as FLT3-D835 point mutations, PML-RAR alpha expression and other predictive factors in 129 APL patients at diagnosis enrolled on the Spanish LPA96 (n=43) or LPA99 (n=86) PETHEMA trials.ResultsFLT3-ITDs and D835 mutations were detected in 21% and 9% of patients, respectively. Patients with increased ITD mutant/wild-type ratio or longer ITD size displayed shorter 5-year relapse-free survival (RFS) (P=0.048 and P