Patients with post-essential thrombocythemia and post-polycythemia vera differ from patients with primary myelofibrosis

Lucia Masarova, Prithviraj Bose, Naval Daver, Naveen Pemmaraju, Kate J. Newberry, Taghi Manshouri, Jorge Cortes, Hagop M. Kantarjian, Srdan Verstovsek

Research output: Contribution to journalArticle

Abstract

Prognostic scoring systems for primary myelofibrosis (PMF) are not accurate in patients with post-essential thrombocythemia and post-polycythemia vera myelofibrosis (PET-MF; PPV-MF). Given the paucity of data describing the clinical characteristics, disease course and outcomes of these patients, we sought to describe and compare the clinical characteristics and outcomes of 755 patients with PMF, 181 with PPV-MF, and 163 with PET-MF referred to our institution between 1984 and 2013. The median follow-up was 31 months, and 56% (n = 616) patients had died. Over an observation period of 3502 person-years, 11% of patients had progression to AML, with similar rates among groups. The proportion of patients with transfusion dependency (higher in PMF), leukocytosis and systemic symptoms (higher in PPV-MF), and thrombocytopenia (higher in PMF, PPV-MF) differed among groups. Median overall survival (OS) was longest in PET-MF patients (73 mo vs 45 mo (PMF) vs 48 mo (PPV-MF), p < 0.001). Stratification of OS by DIPSS was only discriminatory in patients with PMF, and it failed to distinguish higher risk patients with PPV/PET-MF. In multivariate analysis, predictors of inferior OS were higher age, anemia, systemic symptoms, thrombocytopenia, and high peripheral blasts in PMF; age, anemia, and systemic symptoms for PPV-MF; and anemia, peripheral blasts and thrombocytopenia in PET-MF. Although the clinical characteristics of PPV/PET-MF patients are not substantially different from those with PMF, their outcomes differ and prognostic scoring systems for PET/PPV-MF should be improved.

Original languageEnglish (US)
Pages (from-to)110-116
Number of pages7
JournalLeukemia Research
Volume59
DOIs
StatePublished - Aug 2017
Externally publishedYes

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Essential Thrombocythemia
Polycythemia Vera
Primary Myelofibrosis
Thrombocytopenia
Anemia
Survival
Leukocytosis
Multivariate Analysis
Observation

Keywords

  • essential thrombocythemia myelofibrosis
  • polycythemia vera myelofibrosis
  • Post
  • Post
  • Primary myelofibrosis
  • Prognosis

ASJC Scopus subject areas

  • Hematology
  • Oncology
  • Cancer Research

Cite this

Masarova, L., Bose, P., Daver, N., Pemmaraju, N., Newberry, K. J., Manshouri, T., ... Verstovsek, S. (2017). Patients with post-essential thrombocythemia and post-polycythemia vera differ from patients with primary myelofibrosis. Leukemia Research, 59, 110-116. https://doi.org/10.1016/j.leukres.2017.06.001

Patients with post-essential thrombocythemia and post-polycythemia vera differ from patients with primary myelofibrosis. / Masarova, Lucia; Bose, Prithviraj; Daver, Naval; Pemmaraju, Naveen; Newberry, Kate J.; Manshouri, Taghi; Cortes, Jorge; Kantarjian, Hagop M.; Verstovsek, Srdan.

In: Leukemia Research, Vol. 59, 08.2017, p. 110-116.

Research output: Contribution to journalArticle

Masarova, L, Bose, P, Daver, N, Pemmaraju, N, Newberry, KJ, Manshouri, T, Cortes, J, Kantarjian, HM & Verstovsek, S 2017, 'Patients with post-essential thrombocythemia and post-polycythemia vera differ from patients with primary myelofibrosis', Leukemia Research, vol. 59, pp. 110-116. https://doi.org/10.1016/j.leukres.2017.06.001
Masarova, Lucia ; Bose, Prithviraj ; Daver, Naval ; Pemmaraju, Naveen ; Newberry, Kate J. ; Manshouri, Taghi ; Cortes, Jorge ; Kantarjian, Hagop M. ; Verstovsek, Srdan. / Patients with post-essential thrombocythemia and post-polycythemia vera differ from patients with primary myelofibrosis. In: Leukemia Research. 2017 ; Vol. 59. pp. 110-116.
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abstract = "Prognostic scoring systems for primary myelofibrosis (PMF) are not accurate in patients with post-essential thrombocythemia and post-polycythemia vera myelofibrosis (PET-MF; PPV-MF). Given the paucity of data describing the clinical characteristics, disease course and outcomes of these patients, we sought to describe and compare the clinical characteristics and outcomes of 755 patients with PMF, 181 with PPV-MF, and 163 with PET-MF referred to our institution between 1984 and 2013. The median follow-up was 31 months, and 56{\%} (n = 616) patients had died. Over an observation period of 3502 person-years, 11{\%} of patients had progression to AML, with similar rates among groups. The proportion of patients with transfusion dependency (higher in PMF), leukocytosis and systemic symptoms (higher in PPV-MF), and thrombocytopenia (higher in PMF, PPV-MF) differed among groups. Median overall survival (OS) was longest in PET-MF patients (73 mo vs 45 mo (PMF) vs 48 mo (PPV-MF), p < 0.001). Stratification of OS by DIPSS was only discriminatory in patients with PMF, and it failed to distinguish higher risk patients with PPV/PET-MF. In multivariate analysis, predictors of inferior OS were higher age, anemia, systemic symptoms, thrombocytopenia, and high peripheral blasts in PMF; age, anemia, and systemic symptoms for PPV-MF; and anemia, peripheral blasts and thrombocytopenia in PET-MF. Although the clinical characteristics of PPV/PET-MF patients are not substantially different from those with PMF, their outcomes differ and prognostic scoring systems for PET/PPV-MF should be improved.",
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AU - Bose, Prithviraj

AU - Daver, Naval

AU - Pemmaraju, Naveen

AU - Newberry, Kate J.

AU - Manshouri, Taghi

AU - Cortes, Jorge

AU - Kantarjian, Hagop M.

AU - Verstovsek, Srdan

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AB - Prognostic scoring systems for primary myelofibrosis (PMF) are not accurate in patients with post-essential thrombocythemia and post-polycythemia vera myelofibrosis (PET-MF; PPV-MF). Given the paucity of data describing the clinical characteristics, disease course and outcomes of these patients, we sought to describe and compare the clinical characteristics and outcomes of 755 patients with PMF, 181 with PPV-MF, and 163 with PET-MF referred to our institution between 1984 and 2013. The median follow-up was 31 months, and 56% (n = 616) patients had died. Over an observation period of 3502 person-years, 11% of patients had progression to AML, with similar rates among groups. The proportion of patients with transfusion dependency (higher in PMF), leukocytosis and systemic symptoms (higher in PPV-MF), and thrombocytopenia (higher in PMF, PPV-MF) differed among groups. Median overall survival (OS) was longest in PET-MF patients (73 mo vs 45 mo (PMF) vs 48 mo (PPV-MF), p < 0.001). Stratification of OS by DIPSS was only discriminatory in patients with PMF, and it failed to distinguish higher risk patients with PPV/PET-MF. In multivariate analysis, predictors of inferior OS were higher age, anemia, systemic symptoms, thrombocytopenia, and high peripheral blasts in PMF; age, anemia, and systemic symptoms for PPV-MF; and anemia, peripheral blasts and thrombocytopenia in PET-MF. Although the clinical characteristics of PPV/PET-MF patients are not substantially different from those with PMF, their outcomes differ and prognostic scoring systems for PET/PPV-MF should be improved.

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