Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2024 Jan;103(1):97-103.
doi: 10.1007/s00277-023-05528-4. Epub 2023 Nov 10.

Real-world clinical characteristics of post-essential thrombocythemia and post-polycythemia vera myelofibrosis

Kotaro Shide  1 , Katsuto Takenaka  2 , Akira Kitanaka  3 , Akihiko Numata  4 , Takuro Kameda  1 , Takuji Yamauchi  4 , Atsushi Inagaki  5 , Shohei Mizuno  6 , Akiyoshi Takami  6 , Shinichi Ito  7 , Masao Hagihara  8 , Kensuke Usuki  9 , Takaaki Maekawa  10 , Kazutaka Sunami  11 , Yasunori Ueda  12 , Miyuki Tsutsui  13 , Miki Ando  13 , Norio Komatsu  13 , Keiya Ozawa  14 , Mineo Kurokawa  15 , Shunya Arai  15 , Kinuko Mitani  16 , Koichi Akashi  4 , Kazuya Shimoda  17
Affiliations

Real-world clinical characteristics of post-essential thrombocythemia and post-polycythemia vera myelofibrosis

Kotaro Shide et al. Ann Hematol. 2024 Jan.

Abstract

There are few prospective studies on patients with post-essential thrombocythemia myelofibrosis (PET-MF) and post-polycythemia vera myelofibrosis (PPV-MF). Therefore, we conducted a nationwide longitudinal prospective survey to clarify the clinical characteristics of these diseases. A total of 197 PET-MF and 117 PPV-MF patients diagnosed between 2012 and 2021 were analyzed. The median age at diagnosis was 70.0 years for both diseases. The time from diagnosis of ET or PV to that of MF was 9.6 and 10.4 years, respectively, with no significant difference. Patients with PPV-MF had higher hemoglobin levels and white blood cell counts than those with PET-MF, whereas those with PET-MF had higher platelet counts than those with PPV-MF. Although splenomegaly was more frequent in patients with PPV-MF at diagnosis, there was no difference in the frequency of constitutional symptoms. Ruxolitinib was the most common treatment administered to 74.6% and 83.8% of patients with PET-MF and PPV-MF, respectively. Patients with PET-MF and PPV-MF had similar prognoses, with 3-year overall survival (OS) of 0.742 in PET-MF and 0.768 in PPV-MF patients. In both diseases, leukemic transformation was the leading cause of death, followed by infection. The 3-year OS for patients with PET/PPV-MF and primary MF diagnosed during the same period was 0.754 and 0.626, respectively, with no significant difference. This survey provides real-world clinical features and prognostic data on secondary myelofibrosis in the ruxolitinib era.

Keywords: Post-essential thrombocythemia myelofibrosis; Post-polycythemia vera myelofibrosis; Primary myelofibrosis; Ruxolitinib.

PubMed Disclaimer

References

    1. Barbui T, Barosi G, Birgegard G, Cervantes F, Finazzi G, Griesshammer M, Harrison C, Hasselbalch HC, Hehlmann R, Hoffman R, Kiladjian JJ, Kroger N, Mesa R, McMullin MF, Pardanani A, Passamonti F, Vannucchi AM, Reiter A, Silver RT, Verstovsek S, Tefferi A, European L (2011) Philadelphia-negative classical myeloproliferative neoplasms: critical concepts and management recommendations from European LeukemiaNet. J Clin Oncol 29(6):761–770. https://doi.org/10.1200/JCO.2010.31.8436 - DOI - PubMed - PMC
    1. Thiele J, Kvasnicka HM, Orazi A, Tefferi A, Bigegard G, Barbui T (2017) Polycythaemia vera. In: Swerdlow SH, Campo E, Harris NL et al (eds) WHO classification of tumors of haematopoietic and lymphoid tissues. IARC press, Lyon, pp 39–43
    1. Thiele J, Kvasnicka HM, Orazi A, Gianeli U, Tefferi A, Gisslinger H, Barbui T (2017) Essential thrombocythaemia. In: Swerdlow SH, Campo E, Harris NL et al (eds) WHO classification of tumors of haematopoietic and lymphoid tissues. IARC press, Lyon, pp 50–53
    1. Thiele J, Kvasnicka HM, Orazi A, Gianeli U, Barbui T, Barosi G, Tefferi A (2017) Primary myelofibrosis. In: Swerdlow SH, Campo E, Harris NL et al (eds) WHO classification of tumors of haematopoietic and lymphoid tissues. IARC press, Lyon, pp 44–50
    1. Cerquozzi S, Tefferi A (2015) Blast transformation and fibrotic progression in polycythemia vera and essential thrombocythemia: a literature review of incidence and risk factors. Blood Cancer J 5:e366. https://doi.org/10.1038/bcj.2015.95 - DOI - PubMed - PMC

MeSH terms

LinkOut - more resources