Philadelphia chromosome-like acute lymphoblastic leukemia: progress in a new cancer subtype.

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Philadelphia chromosome-like acute lymphoblastic leukemia: progress in a new cancer subtype.

Clin Adv Hematol Oncol. 2017 Jul;15(7):554-561

Authors: Wells J, Jain N, Konopleva M

Abstract
Philadelphia chromosome-like (Ph-like) acute lymphoblastic leukemia (ALL) is a newly described, high-risk subtype of B-cell ALL. It is characterized by a gene expression profile similar to that of Ph-positive ALL; however, the BCR-ABL1 fusion is not present. The World Health Organization classification of myeloid neoplasms and acute leukemia recently was updated to include the Ph-like or BCR-ABL1-like ALL subtype of B-cell ALL as a provisional entity. Unlike Ph-positive ALL, which is characterized by the pathognomonic BCR-ABL1 fusion, Ph-like ALL is characterized by a multitude of different genetic rearrangements and mutations. In this review, we outline the age-related and geographic incidence of Ph-like ALL, the association with worse clinical outcomes, and early evidence for the use of ruxolitinib (a Janus kinase 2 inhibitor) and dasatinib (a tyrosine kinase inhibitor targeting ABL1).

PMID: 28749919 [PubMed - indexed for MEDLINE]

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