Selected cell receptor genotypes differentially modulate the ABO blood group influence on Factor VIII levels in severe aortic stenosis

Selected cell receptor genotypes differentially modulate the ABO blood group influence on Factor VIII levels in severe aortic stenosis

Barbara Lunghi a); Annalisa Castagna b); Alessio Branchini a); Marcello Baroni a); Giovanna Marchetti c); Gianni Mazzoni c); Cristina Legnani d); Marianna Spizzo b); Gabriele Mango b); Simonetta Friso b) Nicola Martinelli b),1); Francesco Bernardi a),1).

a) Department of Life Sciences and Biotechnology, University of Ferrara, Italy
b) Department of Medicine, University of Verona, Italy
c) Department of Neuroscience and Rehabilitation, University of Ferrara, Italy
d) Fondazione Arianna Anticoagulazione, Bologna, Italy
1) F.B. and N.M. contributed equally to this work as senior authors.

Abstract

Introduction

There are few large-scale, population-based studies detailing the risks of thrombosis, hemorrhage, leukemic transformation in patients with myeloproliferative neoplasms (MPNs), including essential thrombocythemia (ET), polycythemia vera (PV), and primary myelofibrosis (PMF).

Methods

We performed a nationwide longitudinal cohort study using the Korean National Health Insurance System (NHIS) database. MPN patients (n = 11,991) and their 1:4 age- and sex-matched controls (n = 47,964) were enrolled. The risk of thrombosis, hemorrhage, leukemic transformation was estimated using a Cox proportional hazards regression, and stratified analyses were performed for related factors.

Results

During a median of 7.8 years of follow-up, 30.1 % of MPN patients (3614/11,991) and 19.0 % of the matched controls (9141/47,964) developed arterial thrombosis, 11.6 % of MPN patients (1397/11,991) and 6.4 % of the matched controls (3099/47,964) developed venous thrombosis and 18.7 % of MPN patients (2251/11,991) and 12.1 % of the matched controls (5836/47,964) developed hemorrhage. 4.9 % of MPN patients (597/11,991) and 0.1 % of matched controls (50/47,964) developed leukemia. The overall risk of developing thrombosis, hemorrhage, leukemic transformation was higher in MPN patients (adjusted hazard ratio [aHR] 1.695, 95 % confidence interval [CI]: 1.629–1.765 for arterial thrombosis, aHR 1.963, 95 % CI: 1.838–2.096 for venous thrombosis, and aHR 1.714, 95 % CI: 1.630–1.802 for hemorrhage) than in the controls. Patients with MPNs had a 10-year cumulative incidence of leukemic transformation of 6.2 %.

Conclusion

The patients with MPNs have a higher risk of thrombosis, hemorrhage, and leukemic transformation than matched controls. Strategies are warranted to reduce the risk of thrombosis, hemorrhage, and leukemic transformation in MPN patients.