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Current Opinion in Hematology:
doi: 10.1097/MOH.0000000000000050
LYMPHOID BIOLOGY AND DISEASES: Edited by Ari M. Melnick

Beyond Philadelphia: ‘Ph-like’ B cell precursor acute lymphoblastic leukemias – diagnostic challenges and therapeutic promises

Izraeli, Shaia,b,c

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Abstract

Purpose of review

The presence of the Philadelphia chromosome causing the fusion between BCR to ABL1 in B cell precursor acute lymphoblastic leukemias (ALLs) was associated with a particularly bad prognosis, which has been markedly improved with the addition of imatinib to chemotherapy. Recent genomic studies have lead to the identification of ‘Philadelphia like’ or ‘BCR-ABL1 like’ ALLs lacking BCR-ABL1 fusion.

Recent findings

About 10% of childhood ALL and a higher percentage of adolescents and adults with ALLs are characterized by activation of cytokine receptors and signaling kinases. Aberrant expression, point mutations or fusion translocations cause activation of either the ABL1 or JAK signaling pathways. In general, these leukemias are associated with worse prognosis. Preclinical studies and limited clinical experience suggest that these leukemias respond to tyrosine kinase inhibitors. Thus, their identification is important. However, as most of these fusion translocations are rare, their diagnosis is challenging.

Summary

The diagnosis of ‘Philadelphia like’ poor prognosis ALLs is technically challenging but of paramount importance as they are likely to respond to targeted therapy with currently available ABL or JAK inhibitors.

© 2014 Wolters Kluwer Health | Lippincott Williams & Wilkins

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