| Title | Acute promyelocytic leukemia in childhood. | | Author(s) | Gregory J, Feusner J | | Institution | Department of Pediatric Hematology/Oncology,Goryeb Children's Hospital, 100 Madison Avenue, Morristown, NJ 07962, USA. john.gregoryjr@atlantichealth.org. | | Source | Curr Oncol Rep 2009 Nov; 11(6):439-45. | | 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)-alpha that results from a balanced reciprocal translocation between the PML gene on chromosome 15 and the RAR-alpha (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. | | Language | eng | | Pub Type(s) | Journal Article
| | PubMed ID | 19840521 |
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