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Chronic myeloproliferative neoplasms

Normal life expectancy for polycythemia vera (PV) patients is possible

Leukemia volume 36, pages 569–572 (2022)Cite this article

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Polycythemia vera (PV) is a chronic myeloproliferative neoplasm (MPN) characterized by shortened survival due to potentially fatal complications including thrombosis, and progression to myelofibrosis (MF) or acute myeloid leukemia (AML) [1]. Historically, thrombosis has been the major contributor to early mortality and the leading cause of death in PV [2]. Vigilant phlebotomy to a target hematocrit [3], low dose aspirin [4], and myelosuppressive therapy [2] have all reduced fatal cardiovascular events and major thromboses. This has led to treatment recommendations from European LeukemiaNet (ELN) and National Comprehensive Cancer Network (NCCN) focused on preventing thrombosis [5, 6]. Despite this success, overall survival (OS) of PV patients remains shortened compared to the general population [7]. PV progression to MF or AML is a frequent cause of late morbidity, and is likely to be the leading cause of excess mortality when thrombosis is prevented [1, 8]. As such, current recommendations which focus on thrombosis prevention may not satisfy the needs of younger, “low-risk”, patients harboring a lifetime threat of progression [9, 10]. Because there are no established recommendations to prevent progression, the OS of PV patients most likely remains shortened. Our objective was to assess the extent by which PV alters the life expectancy of patients receiving available treatment, and to determine whether these patients can have a normal life expectancy. We therefore compared the OS of PV patients treated at our academic medical center, and PV patients in the United States (US), to the expected survival of the general US population.

To assess PV survival in the context of modern diagnostic criteria, risk stratification, and treatment guidelines, we obtained PV survival data from our Weill Cornell Medicine (WCM) Research Database Repository (RDR) and from the National Cancer Institute Surveillance, Epidemiology and End Results (SEER) Program. WCM institutional review board approval and SEER data use agreement were in place. The WCM RDR contains PV survival data through data closure, April 2020 [9]. PV diagnosis at WCM was established using PVSG criteria (1974–2007), WCM criteria (2008–2015), or WHO 2016 criteria (2016–2020) (Supplementary references 2–4). SEER classifies cancer by the third edition of the International Classification of Diseases for Oncology (ICD-O-3) and aligns closely with the World Health Organization (WHO) definitions. In 2001, PV became reportable to US cancer registries available in SEER. The SEER 18 database used for this study includes cancer registries from 18 states, covering ~35% of the US population from 2000 through 2017. Statistical analyses and plots were done using RStudio software v 1.4.1106 (Supplementary references).

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Fig. 1: Demographics and survival analysis of PV-WCM and SEER patients.
Fig. 2: Cumulative incidence of PV progression to MF.

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Acknowledgements

This study was supported by grants from the David L. Johns Family of the Cancer Research & Treatment Fund (CR&T) and MPN Peoria.

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  1. Myeloproliferative Neoplasms (MPN) Center, Division of Hematology and Oncology, Weill Cornell Medicine, New York, NY, USA

    Ghaith Abu-Zeinah, Richard T. Silver & Joseph M. Scandura

  2. School of Clinical Medicine, University of Cambridge, Cambridge, UK

    Khalid Abu-Zeinah

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  1. Ghaith Abu-Zeinah
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GAZ/JMS/RTS wrote the manuscript. GAZ/JMS designed and performed the statistical analysis. KAZ collected and analyzed data.

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Correspondence to Ghaith Abu-Zeinah.

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Competing interests

GAZ—Advisory board, PharmaEssentia. RTS—Speaker bureau and consultant, PharmaEssentia. KAZ—None. JMS—Research support and advisory board, Abbvie.

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Abu-Zeinah, G., Silver, R.T., Abu-Zeinah, K. et al. Normal life expectancy for polycythemia vera (PV) patients is possible. Leukemia 36, 569–572 (2022). https://doi.org/10.1038/s41375-021-01447-3

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