International Journal of Hematology

, Volume 105, Issue 1, pp 59–69 | Cite as

Clinical features and outcomes of patients with primary myelofibrosis in Japan: report of a 17-year nationwide survey by the Idiopathic Disorders of Hematopoietic Organs Research Committee of Japan

  • Katsuto Takenaka
  • Kazuya Shimoda
  • Naoyuki Uchida
  • Taizo Shimomura
  • Koji Nagafuji
  • Tadakazu Kondo
  • Hirohiko Shibayama
  • Takehiko Mori
  • Kensuke Usuki
  • Taichi Azuma
  • Yutaka Tsutsumi
  • Junji Tanaka
  • Hitomi Dairaku
  • Keitaro Matsuo
  • Keiya Ozawa
  • Mineo Kurokawa
  • Shunya Arai
  • Koichi AkashiEmail author
Original Article


We conducted a 17-year nationwide survey (1999–2015) to elucidate the clinical outcomes of patients with primary myelofibrosis (PMF) in Japan. Questionnaires were sent annually to approximately 500 hematology departments. Newly diagnosed patients with PMF were enrolled in this study, and were followed up annually to collect prognostic information. Approximately 50 patients were enrolled per year, yielding a total of 780 patients with PMF included in this study. The median age at diagnosis was 66 years. At the time of analysis, the median survival duration was 47 months, and the 3-year overall survival rate was 59 %. Infection and disease transformation into acute leukemia were the most frequent causes of death. Of the proposed prognostic models for predicting the outcomes of PMF patients in Japan, the Dynamic International Prognostic Scoring System of PMF plus model was the most feasible. Forty-three patients received allogeneic hematopoietic stem cell transplantation (alloSCT) at a median of 343 days after diagnosis. This treatment significantly prolonged the survival of PMF patients, and the 3-year overall survival rate after first alloSCT was 84 %. A long-term registration study is required for further evaluation of prognosis and the impact of treatments on survival.


Myelofibrosis Nationwide survey Long-term outcome 



This study was supported by a research grant from the Idiopathic Disorders of Hematopoietic Organs Research Committee of the Ministry of Health, Labor and Welfare of Japan (H23-Nanchi-Ippan-001, H26-Nanchi-Ippan-062, H27-Nanchi-Ippan-017, a Grant-in-Aid for Scientific Research (C) (to K.T., No. 26461424), and a Grant-in-Aid for Scientific Research (S) (to K.A., No. 16H06391). The authors are grateful to their collaborators for their valuable contributions and acknowledge all the institutions that participated in this study and are listed in the supplementary document.

Compliance with ethical standards

Conflict of interest

The authors declare that no conflicts of interest exist.

Supplementary material

12185_2016_2102_MOESM1_ESM.docx (17 kb)
Figure S1. Number of years of rainfall series for each station (DOCX 17 kb)


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Copyright information

© The Japanese Society of Hematology 2016

Authors and Affiliations

  • Katsuto Takenaka
    • 1
  • Kazuya Shimoda
    • 2
  • Naoyuki Uchida
    • 3
  • Taizo Shimomura
    • 4
  • Koji Nagafuji
    • 5
  • Tadakazu Kondo
    • 6
  • Hirohiko Shibayama
    • 7
  • Takehiko Mori
    • 8
  • Kensuke Usuki
    • 9
  • Taichi Azuma
    • 10
  • Yutaka Tsutsumi
    • 11
  • Junji Tanaka
    • 12
  • Hitomi Dairaku
    • 1
  • Keitaro Matsuo
    • 13
  • Keiya Ozawa
    • 14
  • Mineo Kurokawa
    • 15
  • Shunya Arai
    • 15
  • Koichi Akashi
    • 1
    Email author
  1. 1.Department of Medicine and Biosystemic ScienceKyushu University Graduate School of Medical SciencesFukuokaJapan
  2. 2.Department of Gastroenterology and Hematology, Faculty of MedicineUniversity of MiyazakiMiyazakiJapan
  3. 3.Department of HematologyToranomon HospitalTokyoJapan
  4. 4.Department of HematologyKumamoto Shinto General HospitalKumamotoJapan
  5. 5.Division of Hematology and Oncology, Department of MedicineKurume University School of MedicineKurumeJapan
  6. 6.Department of Hematology and Oncology, Graduate School of MedicineKyoto UniversityKyotoJapan
  7. 7.Department of Hematology and OncologyOsaka University Graduate School of MedicineOsakaJapan
  8. 8.Division of Hematology, Department of MedicineKeio UniversityTokyoJapan
  9. 9.Department of HematologyNTT Medical Center TokyoTokyoJapan
  10. 10.Department of Hematology, Clinical Immunology, and Infectious DiseasesEhime University Graduate School of MedicineEhimeJapan
  11. 11.Department of HematologyHakodate Municipal HospitalHokkaidoJapan
  12. 12.Department of HematologyTokyo Women’s Medical UniversityTokyoJapan
  13. 13.Division of Molecular MedicineAichi Cancer Center Research InstituteNagoyaJapan
  14. 14.The Institute of Medical ScienceThe University of TokyoTokyoJapan
  15. 15.Department of Hematology and Oncology, Graduate School of MedicineThe University of TokyoTokyoJapan

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