Ph-Like ALL: Diagnosis and Management

Abstract

Philadelphia chromosome-like acute lymphoblastic leukemia (Ph-like or BCR-ABL1-like ALL) is a recently described subset of B-ALL occurring in children, adolescents and young adults (AYAs), and older adults. Ph-like ALL has a gene expression profile similar to that of Philadelphia chromosome-positive (Ph+) ALL, but without the characteristic BCR-ABL1 fusion oncogene expressed from t(9;22)(q34.1;q11.2). Similar to Ph+ ALL, Ph-like ALL is associated with a kinase-activated gene expression signature and frequent concomitant deletions of IKZF1, a gene encoding the lymphoid transcription factor IKAROS, as well as with high relapse risk and poor clinical outcomes. In this chapter, we discuss the biologic and clinical features of Ph-like ALL and describe the current and planned precision medicine trials for these high-risk patients.