Adults with Philadelphia chromosome-like acute lymphoblastic leukemia frequently have IGH-CRLF2 and JAK2 mutations, persistence of minimal residual disease and poor prognosis

Adults with Philadelphia chromosome-like acute lymphoblastic leukemia frequently have IGH-CRLF2 and JAK2 mutations, persistence of minimal residual disease and poor prognosis
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DOI:
10.3324/haematol.2015.136366
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发表时间:
2017-01-01
期刊:
影响因子:
10.1
通讯作者:
Goekbuget, Nicola
Goekbuget, Nicola
中科院分区:
医学1区
文献类型:
--
作者:
Herold, Tobias;Schneider, Stephanie;Goekbuget, Nicola

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费城样b细胞前体急性淋巴母细胞白血病(Ph-like ALL)在儿童和年轻人中具有明显的遗传改变和预后差的特点。本研究的目的是研究成人ph样ALL的遗传和临床特征。在207例患有b细胞前体ALL (BCP-ALL)的成人患者(中位年龄:42岁)中,26例(13%)通过基因表达谱被归类为ph样ALL。在95例BCR-ABL1和kmt2a重排阴性的BCP-ALL患者中,ph样ALL的发生率为27%。IGH-CRLF2重排(6/16;P=0.002)和JAK2突变(7/16;P=0.002
Philadelphia-like B-cell precursor acute lymphoblastic leukemia (Ph-like ALL) is characterized by distinct genetic alterations and inferior prognosis in children and younger adults. The purpose of this study was a genetic and clinical characterization of Ph-like ALL in adults. Twenty-six (13%) of 207 adult patients (median age: 42 years) with B-cell precursor ALL (BCP-ALL) were classified as having Ph-like ALL using gene expression profiling. The frequency of Ph-like ALL was 27% among 95 BCP-ALL patients negative for BCR-ABL1 and KMT2A-rearrangements. IGH-CRLF2 rearrangements (6/16; P=0.002) and mutations in JAK2 (7/16; P