Abstract
Introduction: Myeloproliferative neoplasms (MPNs), including polycythemia vera (PV), essential thrombocytosis (ET) and myelofibrosis (MF), are clonal stem cell neoplasms. Therapy is instituted to prevent thrombosis, a leading cause of morbidity/mortality in this population. Atrial fibrillation (AF) is also associated with increased risk of thromboembolism. Risk scores, including CHA 2 DS 2 VASC, are used to determine benefit of anticoagulation (AC) but have not been validated in patients with MPN. Methods: This is a single-center, retrospective cohort study of patients with concurrent MPN and AF from 2000 to 2020 (n = 142; 62 ET, 54 PV, 26 MF). Outcomes included composite of arterial thrombotic events (ATE; MI, ischemic stroke, or peripheral arterial embolism) and cardiovascular (CV) death, bleeding requiring hospitalization, and all-cause mortality (ACM). Results: AC was used in 69% of all MPN patients (73% of ET/ PV, 50% of MF). CHA 2 DS 2 VASC scores were significantly higher in patients with ET/PV compared with MF (4 ± 2 vs 3 ± 1, p = 0.0019). After median follow-up (FU) of 49 months (IQR 18, 86) the composite outcome occurred in 39% of patients with MPN (ET/PV 36%, MF 50%). Bleeding occurred in 30% of patients (ET/PV 29%, MF 30%) and ACM in 45% of all patients (ET/PV 40%, MF 83%). Patients with MF had worse composite-free survival (log-rank p = 0.0043, Fig-A) and no difference in bleed-free survival (Fig-B) compared with ET/PV patients. After multivariable logistic regression including CHA 2 DS 2 VASC, age, sex, MF, AC, CAD, hypertension, diabetes, prior stroke, aspirin use, MPN treatment and FU time as co-variables, MF was associated with the composite (aOR 4.1, 95% CI 1.2-14.9), CHA 2 DS 2 VASC was not (aOR 1.3, 95% CI 0.8-2.1). Conclusions: Patients with MPN and AF have high rates of adverse outcomes with MF having worse composite-free survival compared to ET/PV. Traditional risk scores may underperform in this patient population underscoring the need for further investigation.
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