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The Current Genomic and Molecular Landscape of Philadelphia-like Acute Lymphoblastic Leukemia.

ABSTRACT: Philadelphia (Ph)-like acute lymphoblastic leukemia (ALL) is a high-risk B-cell Acute Lymphoblastic Leukemia (B-ALL) characterized by a gene expression profile similar to Ph-positive B-ALL but lacking the BCR-ABL1 translocation. The molecular pathogenesis of Ph-like B-ALL is heterogenous and involves aberrant genomics, receptor overexpression, kinase fusions, and mutations leading to kinase signaling activation, leukemogenic cellular proliferation, and differentiation blockade. Testing for the Ph-like signature, once only a research technique, is now available to the clinical oncologist. The plethora of data pointing to poor outcomes for this ALL subset has triggered investigations into the role of targeted therapies, predominantly involving tyrosine kinase inhibitors that are showing promising results.

SUBMITTER: Shiraz P

PROVIDER: S-EPMC7139642 | biostudies-literature | 2020 Mar

REPOSITORIES: biostudies-literature

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The Current Genomic and Molecular Landscape of Philadelphia-like Acute Lymphoblastic Leukemia.

Shiraz Parveen P Payne Kimberly J KJ Muffly Lori L

International journal of molecular sciences 20200322 6

Philadelphia (Ph)-like acute lymphoblastic leukemia (ALL) is a high-risk B-cell Acute Lymphoblastic Leukemia (B-ALL) characterized by a gene expression profile similar to Ph-positive B-ALL but lacking the <i>BCR-ABL1</i> translocation. The molecular pathogenesis of Ph-like B-ALL is heterogenous and involves aberrant genomics, receptor overexpression, kinase fusions, and mutations leading to kinase signaling activation, leukemogenic cellular proliferation, and differentiation blockade. Testing fo ...[more]

PMID: 32235787

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