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Early/prefibrotic primary myelofibrosis in patients who were initially diagnosed with essential thrombocythemia

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Abstract

A new entity, namely early/prefibrotic primary myelofibrosis (PMF), was introduced as a subtype of PMF in the 2016 revised World Health Organization (WHO) criteria for myeloproliferative neoplasms (MPN). It was diagnosed based on histopathological features of bone marrow (BM) biopsy specimens together with clinical parameters [leukocytosis, anemia, elevated lactate dehydrogenase (LDH) values, and splenomegaly]. The aim of this study was to evaluate the prevalence of early/prefibrotic PMF in patients who were previously diagnosed with ET, and to compare clinical features at diagnosis and outcomes between early/prefibrotic PMF and essential thrombocythemia (ET) patients. BM biopsy samples obtained at the time of ET diagnosis were available in 42 patients. Sample reevaluation according to the 2016 revised WHO criteria revealed that early/prefibrotic PMF accounted for 14% of patients who were previously diagnosed with ET, which was comparable to the rates in previous reports. Compared to patients with ET, patients with early/prefibrotic PMF had higher LDH values and higher frequencies of splenomegaly. Overall, myelofibrosis-free and acute myeloid leukemia-free survivals were comparable between the 2 groups. Accurate diagnosis is required to clarify the clinical features of Japanese ET patients.

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Authors and Affiliations

  1. Department of Gastroenterology and Hematology, Faculty of Medicine, University of Miyazaki, 5200 Kihara, Kiyotake, Miyazaki, 889-1692, Japan

    Ayako Kamiunten, Kotaro Shide, Takuro Kameda, Masaaki Sekine, Yoko Kubuki, Tomonori Hidaka, Keiichi Akizuki, Yuki Tahira, Haruko Shimoda & Kazuya Shimoda

  2. Japanese Red Cross Nagoya Daiichi Hospital, 3-35 Michishita-cho, Nakamura ku, Nagoya, 453-8511, Japan

    Masafumi Ito

  3. Miyazaki Prefectural Nobeoka Hospital, 2-1-10 Shinkouji, Nobeoka, 882-0835, Japan

    Takanori Toyama

  4. Miyazaki Prefectural Miyazaki Hospital, 5-30 Kitatakamatsu, Miyazaki, 880-8510, Japan

    Noriaki Kawano, Kousuke Marutsuka & Kiyoshi Yamashita

  5. Miyakonojo Medical Center, 5033-1 Iwayoshi-cho, Miyakonojo, 880-8510, Japan

    Kouichi Maeda

  6. Koga General Hospital, 1749-1 Sudaki, Ikeuchi machi, Miyazaki, 880-0041, Japan

    Masanori Takeuchi, Hiroshi Kawano & Hitoshi Matsuoka

  7. Jonan Hospital, 30-5 Daioumachi, Miyakonojo, 885-0026, Japan

    Seiichi Sato

  8. Miyazaki Aisenkai Nichinan Hospital, 3649-2 Kazeta, Nichinan, 887-0034, Japan

    Junzo Ishizaki

Authors
  1. Ayako Kamiunten
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  2. Kotaro Shide
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  3. Takuro Kameda
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  4. Masafumi Ito
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  5. Masaaki Sekine
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  6. Yoko Kubuki
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  7. Tomonori Hidaka
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  8. Keiichi Akizuki
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  9. Yuki Tahira
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  10. Takanori Toyama
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  11. Noriaki Kawano
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  12. Kousuke Marutsuka
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  13. Kouichi Maeda
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  14. Masanori Takeuchi
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  15. Hiroshi Kawano
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  16. Seiichi Sato
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  17. Junzo Ishizaki
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  18. Haruko Shimoda
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  19. Kiyoshi Yamashita
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  20. Hitoshi Matsuoka
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  21. Kazuya Shimoda
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Corresponding author

Correspondence to Kazuya Shimoda.

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Kamiunten, A., Shide, K., Kameda, T. et al. Early/prefibrotic primary myelofibrosis in patients who were initially diagnosed with essential thrombocythemia. Int J Hematol 108, 411–415 (2018). https://doi.org/10.1007/s12185-018-2495-2

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  • DOI: https://doi.org/10.1007/s12185-018-2495-2

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