Acute promyelocytic leukemia in childhood.
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.
Department of Pediatric Hematology/Oncology,Goryeb Children's Hospital, 100 Madison Avenue, Morristown, NJ 07962, USA. john.gregoryjr@atlantichealth.org
John
J
Gregory
James
J
Feusner
D018943:Anthracyclines / Q000627:therapeutic use; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D000077237:Arsenic Trioxide; D001152:Arsenicals / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D002884:Chromosomes, Human, Pair 15; D002886:Chromosomes, Human, Pair 17; D006801:Humans; D015473:Leukemia, Promyelocytic, Acute / Q000188:drug therapy* / Q000235:genetics; D010087:Oxides / Q000627:therapeutic use*; D011379:Prognosis; D014178:Translocation, Genetic; D014212:Tretinoin / Q000627:therapeutic use*
D018943:Anthracyclines; D000970:Antineoplastic Agents; D001152:Arsenicals; D010087:Oxides; D014212:Tretinoin; D000077237:Arsenic Trioxide