Recurrent RARG trinary fusion and ligand binding domain truncation in variant acute promyelocytic leukemia resistant to retinoic acid but sensitive to homoharringtonine‐based therapy

Abstract

Acute promyelocytic leukemia (APL) is a unique and well-defined acute myeloid leukemia (AML) characterized by the PML::RARA fusion caused by t(15;17)(q24.1;q21.2) translocation and the high sensitivity to all-trans retinoic acid (ATRA) and arsenic trioxide (ATO). A minority of AML resembling APL but lacking PML::RARA is called variant APL (vAPL). Patients with vAPL often carry rare v::RARA fusions or fusions involving other retinoic acid receptors RARB and RARG. A recent global study summarized the clinical and laboratory features of 34 RARG fusion positive APL (RARG-APL) cases, including all cases