title: Extramedullary relapse in RARA rearrangement-negative acute promyelocytic leukemia successfully treated in combination with chemotherapy, local radiotherapy, and cord blood transplantation.
abstract: RARA rearrangement-negative acute promyelocytic leukemia (APL) is uncommon, and its extramedullary relapse is extremely rare. We report a 5-year-old girl with RARA rearrangement-negative APL, which recurred solely at the external auditory canal and mastoid air cells. She was successfully treated with chemotherapy, local radiotherapy, and unrelated cord blood transplantation. She has maintained complete remission for 24 months after transplantation. The clinical features and our therapeutic strategy in this patient will provide valuable information for extramedullary relapse of RARA rearrangement-negative APL.
authors.affiliation: *Department of Pediatrics, Sapporo Medical University School of Medicine †Department of Pathology, Teine-Keijinkai Hospital ‡Hokkaido Medical Center for Child Health and Rehabilitation Departments of §Otolaryngology ∥Pathology, JR Sapporo Hospital, Sapporo ¶Department of Pediatrics, Asahikawa Medical University, Asahikawa, Japan.
authors.forename: Keita
authors.initials: K
authors.lastname: Igarashi
authors.forename: Tsukasa
authors.initials: T
authors.lastname: Hori
authors.forename: Masaki
authors.initials: M
authors.lastname: Yamamoto
authors.forename: Natsuko
authors.initials: N
authors.lastname: Inazawa
authors.forename: Hiroko
authors.initials: H
authors.lastname: Noguchi
authors.forename: Nobuhiro
authors.initials: N
authors.lastname: Suzuki
authors.forename: Yukihiro
authors.initials: Y
authors.lastname: Somekawa
authors.forename: Mayumi
authors.initials: M
authors.lastname: Sasaki
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Tsutsumi
authors.forename: Naoki
authors.initials: N
authors.lastname: Hatakeyama
mesh_terms: D064591:Allografts; D000077237:Arsenic Trioxide; D001152:Arsenicals / Q000627:therapeutic use; D003131:Combined Modality Therapy; D036101:Cord Blood Stem Cell Transplantation; D005260:Female; D015321:Gene Rearrangement; D006801:Humans; D007223:Infant; D015473:Leukemia, Promyelocytic, Acute / Q000235:genetics / Q000628:therapy*; D010087:Oxides / Q000627:therapeutic use; D018168:Receptors, Retinoic Acid / Q000235:genetics*; D012008:Recurrence; D000072482:Retinoic Acid Receptor alpha
chemical_list: D001152:Arsenicals; D010087:Oxides; C000607071:RARA protein, human; D018168:Receptors, Retinoic Acid; D000072482:Retinoic Acid Receptor alpha; D000077237:Arsenic Trioxide