title: Characterization of patients with aggressive adult T-cell leukemia-lymphoma in Okinawa, Japan: a retrospective analysis of a large cohort.
abstract: Okinawa Prefecture, located in the subtropics, is an area of endemic adult T-cell leukemia-lymphoma (ATL) in Japan. We retrospectively analyzed 659 patients with aggressive ATL in seven institutions in Okinawa between 2002 and 2011. The median patient age was 68 years. More patients were aged ≥90 years (2.6 %), in this study, than in a nationwide survey (<1 %). The median survival time (MST) of the entire cohort was 6.5 months. Of the 217 patients who had a clinical status similar to that stated in the eligibility criteria of JCOG9801 (a randomized phase III study comparing VCAP-AMP-VECP with CHOP-14), 147 who received the CHOP regimen had a poorer MST than those in the CHOP-14 arm of JCOG9801 (8 vs 11 months). The prevalence of strongyloidiasis in the ATL patients was much higher (12.4 %) than in the historical cohort who visited the University of the Ryukyus Hospital (3.4 %). Furthermore, strongyloidiasis may be associated with ATL-related deaths. These findings suggest that, compared with other areas in Japan, in Okinawa, the proportion of patients aged ≥90 years with clinical features of aggressive ATL is higher, outcomes are poorer, and the disease is associated with a higher prevalence of strongyloidiasis.
authors.affiliation: Division of Endocrinology, Diabetes and Metabolism, Hematology (Second Department of Internal Medicine), Graduate School of Medicine, University of the Ryukyus, Nishihara, Japan.
authors.forename: Yukiko
authors.initials: Y
authors.lastname: Nishi
authors.affiliation: Laboratory of Hematoimmunology, School of Health Sciences, Faculty of Medicine, University of the Ryukyus, 207 Uehara, Nishihara, Okinawa, 903-0215, Japan. fukutaku@med.u-ryukyu.ac.jp.
authors.forename: Takuya
authors.initials: T
authors.lastname: Fukushima
authors.affiliation: Biostatistics Division, Center for Research and Administration and Support, National Cancer Center, Tokyo, Japan.
authors.forename: Shogo
authors.initials: S
authors.lastname: Nomura
authors.affiliation: Division of Endocrinology, Diabetes and Metabolism, Hematology (Second Department of Internal Medicine), Graduate School of Medicine, University of the Ryukyus, Nishihara, Japan.
authors.forename: Takeaki
authors.initials: T
authors.lastname: Tomoyose
authors.affiliation: Division of Endocrinology, Diabetes and Metabolism, Hematology (Second Department of Internal Medicine), Graduate School of Medicine, University of the Ryukyus, Nishihara, Japan.
authors.forename: Sawako
authors.initials: S
authors.lastname: Nakachi
authors.affiliation: Division of Endocrinology, Diabetes and Metabolism, Hematology (Second Department of Internal Medicine), Graduate School of Medicine, University of the Ryukyus, Nishihara, Japan.
authors.forename: Kazuho
authors.initials: K
authors.lastname: Morichika
authors.affiliation: Division of Endocrinology, Diabetes and Metabolism, Hematology (Second Department of Internal Medicine), Graduate School of Medicine, University of the Ryukyus, Nishihara, Japan.
authors.forename: Iori
authors.initials: I
authors.lastname: Tedokon
authors.affiliation: Division of Endocrinology, Diabetes and Metabolism, Hematology (Second Department of Internal Medicine), Graduate School of Medicine, University of the Ryukyus, Nishihara, Japan.
authors.forename: Keita
authors.initials: K
authors.lastname: Tamaki
authors.affiliation: Division of Endocrinology, Diabetes and Metabolism, Hematology (Second Department of Internal Medicine), Graduate School of Medicine, University of the Ryukyus, Nishihara, Japan.
authors.forename: Natsuki
authors.initials: N
authors.lastname: Shimabukuro
authors.affiliation: Department of Hematology, Heartlife Hospital, Nakagusuku, Japan.
authors.forename: Naoya
authors.initials: N
authors.lastname: Taira
authors.affiliation: Department of Hematology, Heartlife Hospital, Nakagusuku, Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Miyagi
authors.affiliation: Department of Hematology, Heartlife Hospital, Nakagusuku, Japan.
authors.forename: Kaori
authors.initials: K
authors.lastname: Karimata
authors.affiliation: Department of Hematology, Heartlife Hospital, Nakagusuku, Japan.
authors.forename: Masayo
authors.initials: M
authors.lastname: Ohama
authors.affiliation: Department of Hematology, Heartlife Hospital, Nakagusuku, Japan.
authors.forename: Atsushi
authors.initials: A
authors.lastname: Yamanoha
authors.affiliation: Department of Hematology, Okinawa Prefectural Chubu Hospital, Uruma, Japan.
authors.forename: Kazumitsu
authors.initials: K
authors.lastname: Tamaki
authors.affiliation: Department of Hematology, Nakagami Hospital, Okinawa, Japan.
authors.forename: Masaki
authors.initials: M
authors.lastname: Hayashi
authors.affiliation: Department of Hematology, Naha City Hospital, Naha, Japan.
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Arakaki
authors.affiliation: Department of Hematology, Naha City Hospital, Naha, Japan.
authors.forename: Jun-Nosuke
authors.initials: JN
authors.lastname: Uchihara
authors.affiliation: Department of Hematology, Okinawa Prefectural Nambu Medical Center and Children's Medical Center, Haebaru, Japan.
authors.forename: Kazuiku
authors.initials: K
authors.lastname: Ohshiro
authors.affiliation: Department of Hematology, Okinawa Red Cross Hospital, Naha, Japan.
authors.forename: Yoshitaka
authors.initials: Y
authors.lastname: Asakura
authors.affiliation: Laboratory of Hematoimmunology, School of Health Sciences, Faculty of Medicine, University of the Ryukyus, 207 Uehara, Nishihara, Okinawa, 903-0215, Japan.
authors.forename: Megumi
authors.initials: M
authors.lastname: Kuba-Miyara
authors.affiliation: Department of Pathology and Cell Biology, Graduate School of Medicine, University of the Ryukyus, Nishihara, Japan.
authors.forename: Kennosuke
authors.initials: K
authors.lastname: Karube
authors.affiliation: Division of Endocrinology, Diabetes and Metabolism, Hematology (Second Department of Internal Medicine), Graduate School of Medicine, University of the Ryukyus, Nishihara, Japan.
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Masuzaki
mesh_terms: D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D015331:Cohort Studies; D003520:Cyclophosphamide / Q000627:therapeutic use; D004317:Doxorubicin / Q000627:therapeutic use; D006801:Humans; D007564:Japan / Q000453:epidemiology; D015459:Leukemia-Lymphoma, Adult T-Cell / Q000150:complications / Q000188:drug therapy / Q000453:epidemiology* / Q000469:parasitology; D011241:Prednisone / Q000627:therapeutic use; D012189:Retrospective Studies; D013322:Strongyloidiasis / Q000209:etiology; D014750:Vincristine / Q000627:therapeutic use
chemical_list: D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D011241:Prednisone
keywords: Adult T-cell leukemia–lymphoma; Okinawa; Retrospective analysis; Strongyloidiasis; Subtropics