Acute promyelocytic leukemia in childhood |
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Authors: | John Gregory James Feusner |
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Institution: | 1. Department of Pediatric Hematology/Oncology, Goryeb Children’s Hospital, 100 Madison Avenue, Morristown, NJ, 07962, USA
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Abstract: | Acute promyelocytic leukemia (APL) is a relatively rare form of acute myelogenous leukemia (AML). In the United States, APL
in children constitutes only 5% to 10% of AML. Molecularly, the disease is characterized by a fusion protein, promyelocytic
leukemia (PML)-retinoic acid receptor (RAR)-α that results from a balanced reciprocal translocation between the PML gene on chromosome 15 and the RAR-α (RARA) gene on chromosome 17. A major advance in the field of APL treatment has been the use of all-trans-retinoic acid (ATRA). Advances in the treatment of APL have taken this form of AML from a disease with significant morbidity
and mortality to one with an excellent outcome. This has resulted largely from the incorporation of ATRA into frontline regimens
with chemotherapy. Anthracyclines remain a cornerstone of treatment at this point. Recent trials have shown a role for arsenic
trioxide in both newly diagnosed and relapsed APL. |
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