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International Journal of Hematology

, Volume 83, Issue 5, pp 443–449 | Cite as

Clinical Features of Polycythemia Vera and Essential Thrombocythemia in Japan: Retrospective Analysis of a Nationwide Survey by the Japanese Elderly Leukemia and Lymphoma Study Group

  • Kazuo Dan
  • Takashi Yamada
  • Yukihiko Kimura
  • Noriko Usui
  • Shinichirou Okamoto
  • Takashi Sugihara
  • Kazue Takai
  • Michihiko Masuda
  • Mayumi Mori
  • Japanese Elderly Leukemia and Lymphoma Study Group
Case Report

Abstract

We conducted the first nationwide survey to clarify the clinical features, treatment methods, and prognoses for polycythemia vera (PV) and essential thrombocythemia (ET). A 1-page questionnaire was mailed to members of the Japanese Elderly Leukemia and Lymphoma Study Group (JELLSG). Surveys on 647 patients (PV, 266 patients; ET, 381 patients) were returned and analyzed. Thrombotic events at diagnosis and during follow-up occurred at rates of 15.4% and 8.5%, respectively, in PV cases and 17.6% and 8.7% in ET cases. Splenomegaly was observed in only 28.8% of PV patients and 10.8% of ET patients. The leukocyte alkaline phosphatase score was elevated in only 46.2% of PV patients. The incidences of abnormal karyotypes were less than 10% in both PV and ET cases. The rates of transformation to myelofibrosis were 2.6% in both PV and ET cases, and acute leukemia was noted in 1.1% of PV patients and 2.9% of ET patients. Prognostic factors were thrombotic history for PV and thrombotic history and age (≥60 years) for ET. The present study clearly demonstrated clinical differences between Japanese and Western patients for PV and ET. Concerning the treatment of PV and ET, the study revealed considerable variation among Japanese hematologists. These results suggest the necessity of developing treatment guidelines according to risk stratification that are suitable for Japanese PV and ET patients.

Key words

Polycythemia vera Essential thrombocythemia Clinical features Nationwide survey Retrospective analysis 

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Copyright information

© The Japanese Society of Hematology 2006

Authors and Affiliations

  • Kazuo Dan
    • 1
  • Takashi Yamada
    • 1
  • Yukihiko Kimura
    • 2
  • Noriko Usui
    • 3
  • Shinichirou Okamoto
    • 4
  • Takashi Sugihara
    • 5
  • Kazue Takai
    • 6
  • Michihiko Masuda
    • 7
  • Mayumi Mori
    • 8
  • Japanese Elderly Leukemia and Lymphoma Study Group
  1. 1.Department of HematologyNippon Medical SchoolTokyoJapan
  2. 2.First Department of Internal MedicineTokyo Medical UniversityTokyoJapan
  3. 3.Department of Hematology/OncologyThe Jikei University School of MedicineTokyoJapan
  4. 4.Department of HematologyKeio University School of MedicineTokyoJapan
  5. 5.Department of HematologyKawasaki Medical SchoolOkayamaJapan
  6. 6.Department of HematologyNiigata City General HospitalNiigataJapan
  7. 7.Department of HematologyTokyo Women’s Medical UniversityTokyoJapan
  8. 8.Department of HematologyTokyo Metropolitan Geriatric HospitalTokyoJapan

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