Preserving thrombosis and life years in polycythemia vera - start by reading the biology of the disease

Sep 22, 2026

A Swedish nationwide study reported in Haematologica (September 2025) shows that patients with myeloproliferative neoplasms continue to lose life years versus the general population, with polycythemia vera (PV) demonstrating about 1.8 years loss in restricted mean survival at 15 years. Despite being labeled “low risk,” younger patients face decades of clonal proliferation, inflammation, thromboinflammation, and resulting vascular injury, myelofibrosis, and secondary cancers. Evidence suggests early biology-guided therapy, including interferon (notably ropeginterferon alfa-2b) and ruxolitinib, can reduce JAK2V617F allele burden, inflammation (NLR), and thrombosis, enabling more precise long-term endpoints and improved quality of life.

LanguageEnglish

CategoryHealthcare

FormatPDF

Pages7

Reading Time18 min

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Abstract

Introduction

Life-years loss in young MPN patients

Sex-specific differences and clinical implications

Rationale for biology-guided therapy in younger PV

PERSPECTIVE ARTICLE Preserving thrombosis and life years in polycythemia vera: start by reading the biology of the disease Tiziano Barbui,1 Arianna Ghirardi,1 Annalisa Condorelli2 and Marta Sobas3 1 FROM Research Foundation, ASST Papa Giovanni XXIII, Bergamo, Italy; 2 Hematology Division, ASST Papa Giovanni XXIII, Bergamo, Italy and 3 Department of Hematology, Faculty of Medicine, Collegium Medicum in Bydgoszcz, Nicolaus Copernicus University in Toruń, 85-168 Bydgoszcz, Poland Correspondence: T. Barbui [tbarbui@fondazionefrom.it](tbarbui@fondazionefrom.it) [Received:](Received: October 10)[ October 10](Received: October 10), 2025. Accepted: January 27, 2026. Early view: February 5, 2026. [https://doi.org/10.3324/haematol.2025.300028](https://doi.org/10.3324/haematol.2025.300028) ©2026 Ferrata Storti Foundation Published under a CC BY-NC license Abstract The Swedish nationwide study by Leontyeva et al., published in Haematologica in September 2025, revealed that patients with myeloproliferative neoplasms (MPN) continue to lose life years compared with the general population, with polycythemia vera (PV) showing a 1.8-year loss in restricted mean survival at 15 years. Despite being classified as “low risk,” these younger patients lose more life years than age-matched peers. They face decades of exposure to clonal proliferation, inflammation, and thromboinflammation, which contribute to vascular injury, myelofibrosis, and secondary cancers. Evidence suggests that early, biology-guided therapy may modify this trajectory. Interferon (particularly ropeginterferon alfa-2b) andruxolitinib reduce JAK2V617F allele burden, systemic inflammation (as reflected by the neutrophil-to-lymphocyte ratio [NLR]), and thrombosis rates, demonstrating long-term disease-modifying potential. The challenge lies in identifying which younger patients should receive cytoreductive therapy, as these treatments, while effective, may be poorly tolerated or burdensome over decades. Biological markers such as persistent leukocytosis, elevated NLR, rising JAK2V617F variant allele frequency, or high phlebotomy burden can guide treatment decisions more precisely than age alone. Tailoring therapy in younger PV patients according to disease biology and individual tolerance may prevent irreversible complications, improve quality of life, and ultimately reduce the number of life years lost. Introduction The recent Swedish study by Leontyeva et al 1 draws attention to an important and still unresolved issue concerning patients with myeloproliferative neoplasms (MPN) . Using a nationwide cohort and advanced statistical methods, the authors demonstrated that young patients with MPN have a shorter life expectancy than the general population. For polycythemia vera (PV) patients in particular, the loss in restricted mean survival time over 15 years is about 1.8 years, while across all MPN it reaches approximately 4.3 years. The study also provides sex-specific survival curves, showing that men and women have different background life expectancies and, in some cases, different excess mortality rates. As a result, the absolute years of life lost can differ by sex even when hazard ratios are similar. These findings shift the focus from short-term hematologic targets to what truly matters to patients: preserving life years. Interestingly, Leontyeva et al. pointed out that women and men do not lose the same number of years at a given age due to dif- ferences in background life expectancy; the same hazard ratio can represent a greater number of years at risk for women 1 In PV, sex differences in outcomes vary by series, but younger women may experience more venous events at unusual sites (e.g., splanchnic or cerebral), for which aspirin alone is inadequate. In this context, inherited and environmental contributors to thrombosis and, in young women, the added risks posed by hormonal contraceptivesand pregnancy should also be considered. Therefore, it

FAQs

What did the Swedish nationwide study show about life-years loss in polycythemia vera?

Patients with MPN continue to lose life years compared with the general population, and PV shows about a 1.8-year loss in restricted mean survival over 15 years.

Why might younger PV patients lose more life years even when classified as “low risk”?

They experience prolonged exposure to clonal proliferation, inflammation, and thromboinflammatory injury, which promotes vascular damage, myelofibrosis, and secondary cancers.

How can early biology-guided therapy potentially change the disease trajectory in younger PV?

Interferon (especially ropeginterferon alfa-2b) and ruxolitinib reduce JAK2V617F allele burden, systemic inflammation (NLR), and thrombosis rates, supporting long-term disease-modifying potential.

Sep 22, 2026

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