title: Successful all-trans retinoic acid treatment of acute promyelocytic leukemia in a patient with NPM/RAR fusion.
abstract: Acute promyelocytic leukemia (APL) is characterized by a reciprocal chromosomal translocation involving the gene for retinoic acid receptor alpha(RAR). Most APL patients have a t(15;17) translocation that generates the PML-RAR fusion gene, and such patients respond well to treatment with all-trans retinoic acid (ATRA). Some APL cases also involve rearrangements that fuse RAR to partner genes other than PML, including nucleophosmin (NPM), promyelocytic leukemia zinc finger (PLZF), nuclear mitotic apparatus (NUMA), and Stat5b, but the clinical characteristics of APL without PML-RAR have not been fully clarified. We describe a 64-year-old man with NPM-RAR-positive APL who was receiving hemodialysis therapy for chronic uremia. Complete remission was achieved with ATRA monotherapy and was maintained for 18 months with consolidation chemotherapy. These findings suggest that ATRA can be used to treat APL patients with NPM/RAR as well as APL with PML/RAR.
authors.affiliation: Division of Hematology, Niigata University Medical and Dental Hospital, Niigata, Japan. okazuka@med.niigata-u.ac.jp
authors.forename: Kiyoshi
authors.initials: K
authors.lastname: Okazuka
authors.forename: Masayoshi
authors.initials: M
authors.lastname: Masuko
authors.forename: Yoshinobu
authors.initials: Y
authors.lastname: Seki
authors.forename: Hitomi
authors.initials: H
authors.lastname: Hama
authors.forename: Noriyuki
authors.initials: N
authors.lastname: Honma
authors.forename: Tatsuo
authors.initials: T
authors.lastname: Furukawa
authors.forename: Ken
authors.initials: K
authors.lastname: Toba
authors.forename: Kenji
authors.initials: K
authors.lastname: Kishi
authors.forename: Yoshifusa
authors.initials: Y
authors.lastname: Aizawa
mesh_terms: D000970:Antineoplastic Agents / Q000008:administration & dosage*; D002884:Chromosomes, Human, Pair 15 / Q000235:genetics; D002886:Chromosomes, Human, Pair 17 / Q000235:genetics; D006801:Humans; D015473:Leukemia, Promyelocytic, Acute / Q000150:complications / Q000188:drug therapy* / Q000235:genetics; D008297:Male; D008875:Middle Aged; D009363:Neoplasm Proteins / Q000235:genetics; D015514:Oncogene Proteins, Fusion / Q000235:genetics; D012074:Remission Induction; D006435:Renal Dialysis; D014178:Translocation, Genetic; D014212:Tretinoin / Q000008:administration & dosage*; D014511:Uremia / Q000150:complications / Q000235:genetics / Q000628:therapy
chemical_list: D000970:Antineoplastic Agents; C120276:NPM-RARalpha protein, human; D009363:Neoplasm Proteins; D015514:Oncogene Proteins, Fusion; C118318:PLZF-RARalpha fusion protein, human; D014212:Tretinoin