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Thromb Haemost
DOI: 10.1055/a-2767-0451
Review Article

Atrial Fibrillation in Philadelphia Chromosome–negative Myeloproliferative Neoplasms: Thromboinflammatory Crosstalk with a Focus on the NLRP3 Inflammasome

Authors

  • Olga Demska 

    1   Department of Hematology, Collegium Medicum in Bydgoszcz, Nicolaus Copernicus University in Torun, Bydgoszcz, Poland

  • Marta Szandruk-Bender 

    2   Department of Pharmacology, Faculty of Medicine, Wroclaw Medical University, Wroclaw, Poland

  • Niels Eske Bruun 

    3   Department of Cardiology, Zealand University Hospital, Roskilde, Denmark

  • Vibe Skov 

    4   Department of Hematology, Zealand University Hospital, Roskilde, Denmark

  • Lasse Kjær 

    4   Department of Hematology, Zealand University Hospital, Roskilde, Denmark

  • Morten Kranker Larsen 

    4   Department of Hematology, Zealand University Hospital, Roskilde, Denmark

  • Christina Ellervik 

    5   Faculty of Health and Medical Sciences, University of Copenhagen, Copenhagen, Denmark
    6   Department of Clinical Biochemistry, Zealand University Hospital, Køge, Denmark
    7   Department of Laboratory Medicine, Boston Children's Hospital, Boston, Massachusetts, United States
    8   Department of Pathology, Harvard Medical School, Boston, Boston, Massachusetts, United States

  • Sebastian Szmit 

    9   Department of Cardio-Oncology, Centre of Postgraduate Medical Education, Warsaw, Poland
    10   Cardio-Oncology Unit, Maria Sklodowska-Curie National Research Institute of Oncology, Warsaw, Poland

  • Hans Carl Hasselbalch 

    5   Faculty of Health and Medical Sciences, University of Copenhagen, Copenhagen, Denmark
    6   Department of Clinical Biochemistry, Zealand University Hospital, Køge, Denmark

  • Marta Sobas 

    1   Department of Hematology, Collegium Medicum in Bydgoszcz, Nicolaus Copernicus University in Torun, Bydgoszcz, Poland
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Abstract

The Philadelphia chromosome–negative myeloproliferative neoplasms (MPNs) and atrial fibrillation (AF) share a common proinflammatory and prothrombotic phenotype. Despite this overlap, patients with coexisting MPNs and AF remain undertreated and poorly characterized, with no specific antithrombotic guidelines addressing this dual pathology. Emerging evidence identifies the nucleotide-binding domain (NOD)-like receptor protein 3 (NLRP3) inflammasome as a central and common mediator of vascular diseases. This review explores the pathophysiologic convergence between MPNs and AF, with a focus on the NLRP3 inflammasome and its downstream cytokines (IL-1β, IL-18), as well as neutrophil extracellular traps (NETs), as unifying drivers of thrombosis, atrial remodeling, and clonal propagation. In MPNs, NLRP3 is activated by JAK2-driven inflammation and sterile danger signals, sustaining a cytokine milieu that promotes pyroptosis, fibrosis, and platelet–leukocyte–endothelial interactions. In AF, inflammasome overactivation in cardiomyocytes and fibroblasts contributes to ectopic activity, electrical remodeling, and fibrosis. NETs, which are enhanced by NLRP3, amplify thrombosis and may link the hematologic and cardiovascular components of the association between MPNs and AF. We critically evaluate the translational potential of inflammasome-derived biomarkers and identify NLRP3 inhibition as a promising adjunctive strategy in MPN patients with AF. The review calls for prospective studies to redefine antithrombotic management in this overlooked population, incorporating molecular, inflammatory, and arrhythmogenic risk dimensions.

Keywords

myeloproliferative neoplasms (MPNs) - atrial fibrillation - JAK2V617F mutation thromboinflammation - NLRP3 inflammasome

Contributors' Statement

M.S. and O.D. contributed to the conception and design of the review; O.D. drafted and wrote the manuscript, developed the structure and prepared the figure and table; M.S.-B. contributed to the structure and substantially revised the manuscript; H.C.H. provided critical input and major revisions; V.S., L.K., M.K.L., N.E.B., C.E., and S.S. contributed to editing and critical revision. All authors approved the submitted version and agreed to be accountable for all aspects of the work.




Publication History

Received: 29 August 2025

Accepted after revision: 06 December 2025

Article published online:
23 December 2025

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