b细胞急性淋巴母细胞白血病中CRLF2基因的表达

Wendy Su, Alair Zhao, Jonah Nahoul, Hailey Mendelsohn, Bilal Hamid, Carlos A Tirado
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摘要

目的:b细胞急性淋巴母细胞白血病(B-ALL)是ALL的一个亚群,占ALL病例的75%。B-ALL有多种染色体非整倍体或染色体重排。在5-15%的B-ALL患者中可以看到CRLF2表达的失调,主要通过免疫球蛋白重链(IGH)的相互易位、CRLF2的重排、X和Y染色体PAR1区域的缺失、CRLF2突变以及CRLF2相关通路的突变发生,并在ph样B-ALL中可见。它们与预后不良有关。Blinatumomab是一种可用的免疫疗法,目前有一些正在进行的临床试验用于治疗CRLF2 B-ALL。本文综述了CRLF2在B-ALL中的作用,并总结了有关其分子途径、临床意义、人口统计学发病率、治疗方法和进一步研究领域的文献。
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CRLF2 Gene in B-cell Acute Lymphoblastic Leukemia.

Objectives: B-cell acute lymphoblastic leukemia (B-ALL) is a subset of ALL that comprises 75% of ALL cases. There are a variety of chromosome aneuploidy or chromosomal rearrangements implicated in B-ALL. Deregulation of CRLF2 expression is seen in 5-15% of B-ALL patients and occurs primarily via a reciprocal translocation with immunoglobulin heavy chain (IGH), rearrangements of CRLF2, deletion within the PAR1 region of the X and Y chromosomes, and CRLF2 mutations as well as mutations of the CRLF2-involved pathways and are seen in Ph-like B-ALL. They are associated with a poor prognosis. Blinatumomab is an available immunotherapy, and there are currently a few ongoing clinical trials to treat CRLF2 B-ALL. This review focuses on the role of CRLF2 in B-ALL and summarizes the literature regarding its molecular pathways, clinical significance, incidence rates across demographics, therapies, and areas of further research.

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