title: Acute promyelocytic leukemia in children and adolescents.
abstract: Acute promyelocytic leukemia (APL) is a rare subtype of AML characterized by a reciprocal balanced translocation between chromosomes 15 and 17 that fuses the PML gene with the RARα gene and leads to the leukemic phenotype. Although best described in large clinical trials of adults, APL, like other forms of AML, also occurs in children. The positive outcome of children with APL mirrors the dramatic increase in survival seen in adults since the introduction of all-trans retinoic acid (ATRA). In this paper, we review the diagnosis of APL in children as well as large, retrospective, clinical trial data collected on pediatric APL. We also raise management issues and toxicities that are unique to children.
authors.affiliation: Leukemia Service, Memorial Sloan-Kettering Cancer Center, New York, N.Y., USA.
authors.forename: Eytan M
authors.initials: EM
authors.lastname: Stein
authors.forename: Martin S
authors.initials: MS
authors.lastname: Tallman
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use*; D001151:Arsenic / Q000627:therapeutic use; D002986:Clinical Trials as Topic; D004359:Drug Therapy, Combination; D006801:Humans; D015473:Leukemia, Promyelocytic, Acute / Q000175:diagnosis / Q000188:drug therapy* / Q000235:genetics / Q000401:mortality; D015996:Survival Rate; D014178:Translocation, Genetic; D014212:Tretinoin / Q000627:therapeutic use*
chemical_list: D000970:Antineoplastic Agents; D014212:Tretinoin; D001151:Arsenic