title: Clinical features and prognosis of patients with myelodysplastic syndromes who were exposed to atomic bomb radiation in Nagasaki.
abstract: There is evidence that radiation exposure is a causative factor of myelodysplastic syndromes (MDS). However, little is known about whether radiation exposure is also a prognostic factor of MDS. We investigated the impact of radiation exposure on the prognosis of MDS in Nagasaki atomic bomb survivors using the International Prognostic Scoring System (IPSS) and the revised version (IPSS-R). Subjects were 140 patients with primary MDS diagnosed between 1985 and 2011 and evaluable for IPSS, IPSS-R, and exposure distance. Of those, 31 were exposed at <1.5 km, 35 at 1.5-2.99 km, and 74 at ≥3.0 km. By the end of March 2014, 47 patients (34%) progressed to overt leukemia and 106 (75.7%) died. By comparing with patients exposed at ≥3.0 km, those exposed at <1.5 km had significantly higher frequencies of abnormal chromosome (P = 0.02), intermediate/poor IPSS, and intermediate/poor/very poor IPSS-R cytogenetic category (P = 0.0001, and P < 0.0001, respectively). As with de novo MDS, multivariate Cox regression analyses revealed that cytogenetic abnormalities, IPSS karyotype, and IPSS-R cytogenetics were significantly associated with poor survival, and cumulative incidence of leukemic transformation in MDS among atomic bomb survivors, but exposure distance was not associated with any poor outcomes. These suggest that exposure to the greater dose of atomic bomb radiation is associated with developing poor cytogenetic abnormalities in MDS, which might consequently lead to overt leukemia among atomic bomb survivors.
authors.affiliation: Department of Hematology, Atomic Bomb Disease and Hibakusya Medicine Unit, Atomic Bomb Disease Institute, Nagasaki University, Nagasaki, Japan.|Department of Hematology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan.
authors.forename: Masatoshi
authors.initials: M
authors.lastname: Matsuo
authors.affiliation: Department of Frontier Life Science, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan. masakoiwng@nagasaki-u.ac.jp.
authors.forename: Masako
authors.initials: M
authors.lastname: Iwanaga
authors.affiliation: Division of Scientific Data Registry, Atomic Bomb Disease and Hibakusya Medicine Unit, Atomic Bomb Disease Institute, Nagasaki University, Nagasaki, Japan.
authors.forename: Hisayoshi
authors.initials: H
authors.lastname: Kondo
authors.affiliation: Nagasaki Prefectural Cancer Registry, Nagasaki, Japan.|Department of Epidemiology, Radiation Effects Research Foundation, Nagasaki, Japan.
authors.forename: Midori
authors.initials: M
authors.lastname: Soda
authors.affiliation: Department of Hematology, Japanese Red Cross Nagasaki Genbaku Hospital, Nagasaki, Japan.
authors.forename: Tatsuro
authors.initials: T
authors.lastname: Jo
authors.affiliation: Department of Hematology, Japanese Red Cross Nagasaki Genbaku Hospital, Nagasaki, Japan.
authors.forename: Kensuke
authors.initials: K
authors.lastname: Horio
authors.affiliation: Department of Internal Medicine, St. Francis Hospital, Nagasaki, Japan.
authors.forename: Yumi
authors.initials: Y
authors.lastname: Takasaki
authors.affiliation: Department of Internal Medicine, Nagasaki Harbor Medical Center City Hospital, Nagasaki, Japan.
authors.forename: Yasuhisa
authors.initials: Y
authors.lastname: Kawaguchi
authors.affiliation: Department of Internal Medicine, Nagasaki Harbor Medical Center City Hospital, Nagasaki, Japan.
authors.forename: Hideki
authors.initials: H
authors.lastname: Tsushima
authors.affiliation: Department of Hematology, Nagasaki University Hospital, Nagasaki, Japan.
authors.forename: Yoshitaka
authors.initials: Y
authors.lastname: Imaizumi
authors.affiliation: Department of Hematology, Nagasaki University Hospital, Nagasaki, Japan.
authors.forename: Daisuke
authors.initials: D
authors.lastname: Imanishi
authors.affiliation: Department of Hematology, Atomic Bomb Disease and Hibakusya Medicine Unit, Atomic Bomb Disease Institute, Nagasaki University, Nagasaki, Japan.|Department of Hematology, Nagasaki University Hospital, Nagasaki, Japan.
authors.forename: Jun
authors.initials: J
authors.lastname: Taguchi
authors.affiliation: Department of Hematology, Nagasaki University Hospital, Nagasaki, Japan.
authors.forename: Yasushi
authors.initials: Y
authors.lastname: Sawayama
authors.affiliation: Department of Hematology, Atomic Bomb Disease and Hibakusya Medicine Unit, Atomic Bomb Disease Institute, Nagasaki University, Nagasaki, Japan.|Department of Hematology, Nagasaki University Hospital, Nagasaki, Japan.
authors.forename: Tomoko
authors.initials: T
authors.lastname: Hata
authors.affiliation: Department of Hematology, Atomic Bomb Disease and Hibakusya Medicine Unit, Atomic Bomb Disease Institute, Nagasaki University, Nagasaki, Japan. y-miyaza@nagasaki-u.ac.jp.|Department of Hematology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan. y-miyaza@nagasaki-u.ac.jp.|Department of Hematology, Nagasaki University Hospital, Nagasaki, Japan. y-miyaza@nagasaki-u.ac.jp.
authors.forename: Yasushi
authors.initials: Y
authors.lastname: Miyazaki
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D004190:Disasters / Q000266:history; D018450:Disease Progression; D005260:Female; D049673:History, 20th Century; D006801:Humans; D015994:Incidence; D053208:Kaplan-Meier Estimate; D007938:Leukemia / Q000453:epidemiology / Q000209:etiology; D008297:Male; D008875:Middle Aged; D009190:Myelodysplastic Syndromes / Q000175:diagnosis* / Q000209:etiology* / Q000401:mortality; D054043:Nuclear Weapons; D063868:Patient Outcome Assessment; D012189:Retrospective Studies; D012307:Risk Factors; D013997:Time Factors
keywords: Atomic bomb survivors; myelodysplastic syndromes; prognosis; radiation exposure; therapy-related myeloid neoplasms