The 4th nation-wide study of adult T-cell leukemia/lymphoma (ATL) in Japan: Estimates of risk of ATL and its geographical and clinical features


  • K. Tajima,

    Corresponding author
    • Division of Epidemiology, Aichi Cancer Center Research Institute, 1-1 Kanoko-den, Chikusa-ku, Nagoya 464, Japan
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  • The T- and B-Cell Malignancy Study Group,

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    • This group comprised: (1) members of the Lymphoma Study Group (Chairman: Dr. M. Shimoyama, Chief, Hematology-Oncology and Clinical Cancer Chemotherapy Division, National Cancer Center Hospital), which is a subgroup in “Studies of Multidisciplinary Treatment of Cancer” (Chairman: Dr. K. Suemasu, Director, National Cancer Center Hospital until March, 1988 and Dr. M. Shimoyama, as of April 1988), Grant 62S-1; and (2) others who were requested to participate in the present study and co-operated in the nation-wide survey of ATL cases.

  • Co-Authors

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    • The following is the list of medical practitioners, co-authors of the study, in order of the location of the institution from south to north. K. ARAKI (Dept. of Internal Medicine, Ryukyu University); O. IKEHARA (Okinawa Chubu Hospital); F. TSUZAKI (Kanoya Prefectural Hospital); M. MATSUMOTO (Inst. of Cancer Research, Kagoshima University); S. HANADA and S. HASHIMOTO (Second Dept. of Internal Medicine, Faculty of Medicine, Kagoshima University); H. UNO (Second Dept. of Internal Medicine. Miyazaki Medical College); S. INOUE (Dept. of Dermatology, Miyazaki Medical College); K. TAMURA and R. MASHITA (Miyazaki Prefectural Hospital); R. MIZUTANI (Second Dept. of Internal Medicine, Medical College of Oita); S. KOMADA (Dept. of Dermatology, Medical College of Oita); T. HOSOKAWA (Oita Prefectural Hospital); H. NAITO (Minamata City Hospital); N. KATAYAMA (Yatsushiro Health Insurance General Hospital); K. TAKATSUKI and K. YAMAGUCHI (Second Dept. of Internal Medicine, Kumamoto University Medical School); T. ARAO (Dept. of Dermatology, Kumamoto University Medical School); M. ICHIMARU (Dept. of Clinical Hematology, Atomic Disease Institute, Nagasaki University); K. KINOSHITA (Dept. of Clinical Hematology, National Nagasaki Central Hospital); S. CHIYODA (Japanese Red Cross Nagasaki Atomic Bomb Hospital); Y. MORIUCHI, (Goto Central Hospital)); Y. SHIMAMOTO (Dept. of Internal Medicine, Saga Medical School); H. NATORI (First Dept. of Internal Medicine, Kurume University School of Medicine); M. KOZURU (Kyushu Cancer Center); K. YAMASAKI (Hamanomachi General Hospital); Y. NIHO (First Dept. of Internal Medicine, Faculty of Medicine, Kyushu University); S. ETO (First Dept. of Internal Medicine, University of Occupational and Environmental Health); I. MIYOSHI (Third Dept. of Internal Medicine, Kochi Medical School); T. KONDO (Uwajima City Hospital); R. YOSHIDA (Yawatahama City General Hospital); H. TOKI (Shikoku Cancer Center Hospital); M. KOSAKA (First Dept. of Internal Medicine, University of Tokushima); H. DOHY (Hiroshima Red Cross Hospital and Atomic-bomb Survivors Hospital); I. KIMURA (Second Dept. of Internal Medicine, Okayama University Medical School); S. NAKAYAMA (Kobe General Hospital); A. HORIUCHI (Third Dept. of Internal Medicine, Kinki University School of Medicine); M. TSUKAGUCHI (Sakai Municipal Hospital); H. KAWAGOE (Osaka National Hospital); H. SUGIYAMA (Third Dept. of Internal Medicine); K. YASUNAGA (First Dept. of Internal Medicine, Kansai Medical University); T. KITANI (Research Institute for Microbial Diseases, Osaka University); S. SHIRAKAWA (Second Dept. of Internal Medicine, Mie University School of Medicine); K. TAJIMA and S. TOMINAGA (Division of Epidemiology, Aichi Cancer Center Research Institute); E. MURAKAWA (Prefectural Cancer Center, Niigata Hospital); F. KODAMA (Kanagawa Cancer Center); H. TOKUHIRO (Dept. of Internal Medicine, Kitasato University School of Medicine); H. MIZOGUCHI (Division of Hematology, Tokyo Women's Medical College); M. SHIMOYAMA (Hematology-Oncology and Clinical Cancer Chemotherapy Division, National Cancer Center Hospital); T. TAKAGI (Chiba Cancer Center Hospital); Y. MIURA (Division of Hematology, Jichi Medical School); T. SAI (Iwaki Kyoritsu General Hospital); I. SATO (National Sendai Hospital); C. MIKUNI (National Sapporo Hospital); I. MAEKAWA (Asahikawa Municipal Hospital).


To estimate the annual incidence of adult T-cell leukemia/lymphoma (ATL) by district in Japan, a large-scale nationwide survey of ATL and of non-Hodgkin's lymphoma was performed in 1988. Questionnaires for the registration of ATL and of T-cell and non-T-cell lymphoma were distributed to the physicians in charge of this survey in 1,287 hospitals with 200 or more beds throughout Japan. From the positive rate of anti-HTLV-I antibody in adults, the annual incidence of ATL was estimated at 697, independently of the present survey. In fact, 657 cases (47% of the estimated number), newly diagnosed during the 2 years January 1986 to December 1987, were registered from 191 general hospitals throughout Japan. Major results obtained from the present survey are as follows: (1) among all ATL cases registered, 51% were from Kyushu and 29% were from metropolitan areas (Kanto, Chubu and Kinki) and most, but not all, patients with ATL in the metropolitan areas had come from the ATL-endemic areas and settled in the metropolitan areas; (2) the estimated annual incidence rates of ATL per million adults were 40.4 in males and 26.4 in females in Kyushu, the overall risk of ATL being 1.5 times as high in males as in females; (3) the age-specific incidence rate in Kyushu increased steeply with age until the age of 70, and then decreased markedly in both sexes; (4) the ratio of T-cell versus non-T-cell lymphomas was 2.9 in Kyushu but 0.5 in other districts of Japan, however, this difference regressed to the average for the whole of Japan if ATL cases were excluded; (5) 26.5% of patients with ATL had a family history of cancer, and among these, 14 (8.2%) were ATL, 21 (12.2%) were lymphoma and 17 (9.9%) were hematopoietic malignancies, the incidence of which was markedly higher than in the general population; (6) with regard to clinicopathological features of ATL, there were more advanced cases in south Kyushu than in other districts, however, these differences were not statistically significant. To clarify the chronological changes and geographical variations in the annual incidence of ATL in Japan, continuous systematic nationwide surveillance is necessary and further nation-wide studies are being prepared.