The fusion gene PML-RARA resulting from t(15;17) is the hallmark of which AML subtype?

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Multiple Choice

The fusion gene PML-RARA resulting from t(15;17) is the hallmark of which AML subtype?

The key idea is that the PML-RARA fusion from t(15;17) is the defining abnormality of acute promyelocytic leukemia, which corresponds to the FAB subtype M3. This fusion creates a hybrid protein that disrupts retinoic acid receptor signaling, blocking differentiation at the promyelocyte stage and leading to an accumulation of abnormal promyelocytes. Because this genetic change is specific to this leukemia type, it serves as the hallmark of M3 rather than other AML subtypes. Clinically, this subtype is notable for the risk of coagulopathy but also for its dramatic responsiveness to targeted therapy with all-trans retinoic acid (and arsenic trioxide), which helps differentiate it from other AMLs.

The key idea is that the PML-RARA fusion from t(15;17) is the defining abnormality of acute promyelocytic leukemia, which corresponds to the FAB subtype M3. This fusion creates a hybrid protein that disrupts retinoic acid receptor signaling, blocking differentiation at the promyelocyte stage and leading to an accumulation of abnormal promyelocytes. Because this genetic change is specific to this leukemia type, it serves as the hallmark of M3 rather than other AML subtypes. Clinically, this subtype is notable for the risk of coagulopathy but also for its dramatic responsiveness to targeted therapy with all-trans retinoic acid (and arsenic trioxide), which helps differentiate it from other AMLs.

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