Recent Advances in the Management of Acute Promyelocytic Leukemia

Abstract

APL represents a medical emergency with a high rate of early mortality. It is necessary to start treatment without delay as soon as the diagnosis is suspected based upon cytologic criteria, and before definitive cytogenetic confirmation of the diagnosis has been made. Acute promyelocytic leukemia is the first malignant disease highly curable with targeted therapy directed at a unique molecular abnormality. Acute promyelocytic leukemia is one of the few hematologic diseases that must be recognized under the microscope by the practicing hematologist because early institution of all-trans retinoic acid (ATRA) at the first suspicion of the disease before confirmation of the diagnosis and aggressive blood product support are critical to reduce early mortality. Diagnosis should be confirmed by molecular detection of PML-RARA.fusion (or rare molecular variants), In addition to conventional karyotyping, FISH, and RT-PCR, immunostaining with anti­ PML antibody can be used for rapid diagnosis of APL. ATRA plus anthracycline-based chemotherapy for induction and consolidation followed by maintenance ATRA with low-dose chemotherapy is currently the standard of care. However, the combination of ATRA and arsenic trioxide, with minimal chemotherapy to control leukocytosis, IS very effective therapy for newly diagnosed• patients. This combination may replace conventional approaches for most, if not all, patients in the very near future. Acute promyelocytic leukemia should be considered in any patient with newly diagnosed acute myeloid leukemia because the treatment is urgent and different from all other subtypes.