
MPNs May Increase Risk of Nonthrombotic Cardiovascular Conditions
Key Takeaways
- Patients with MPNs are at increased risk for nonthrombotic conditions, including heart failure and pulmonary hypertension, necessitating improved understanding and management strategies.
- Pulmonary hypertension prevalence in MPNs varies by phenotype, with myelofibrosis showing the highest rates, and JAK2 inhibition may offer therapeutic benefits.
Patients with myeloproliferative neoplasms (MPNs) appear to be at a higher risk of heart failure and pulmonary hypertension, though more research into the links is needed.
It is well known that patients with
In a
Two years ago, Hobbs and colleagues
Still, even as the body of evidence tying pulmonary hypertension and heart failure to MPNs has increased, the underlying pathophysiology connecting the two cardiovascular diseases with MPNs has been under-explored, the authors said.1
In their paper, Hobbs and colleagues reviewed the current scientific literature and offered suggestions for future research.
Turning first to pulmonary hypertension, the investigators said a range of studies have estimated significantly different prevalences of the disease within the MPN population. Prevalence estimates have ranged from 4-58%, they noted, though a large meta-analysis
How best to treat patients with MPN-associated pulmonary hypertension largely remains an open question. Hobbs and colleagues said patients with myelofibrosis, long-standing MPNs, signs of disease progression, or splenomegaly should be screened for pulmonary fibrosis. They said inhibition of Janus kinase 2 (JAK2) may improve patient outcomes, though more research is needed.
Data regarding the prevalence of heart failure in patients with MPNs is more limited, but Hobbs and colleagues said
The investigators said there is a significant gap in the research related to non-thrombotic complications and MPNs, and thus prospective studies are necessary to better understand the true incidence of pulmonary hypertension and heart failure in people with MPNs.1 Such studies could also help elucidate the risk factors and pathophysiological mechanisms underlying the complications, they said.
Hobbs and colleagues concluded that the emerging evidence suggesting that both pulmonary hypertension and heart failure may be associated with an increased risk of hematologic progression in MPNs demonstrates the potential shared pathophysiology between the conditions. They said that shared pathophysiology could be connected with chronic inflammation and activation of the JAK/signal transducer and activator of transcription (STAT) pathway.
Hobbs and colleagues said further research into the interplay between bone marrow, pulmonary vasculature, and cardiac function could help develop novel therapeutic strategies that could benefit both MPNs and cardiovascular disease.
References
1. Leiva O, Liu O, Kanelidis A, et al. Beyond thrombosis: pulmonary hypertension and heart failure in patients with myeloproliferative neoplasms: JACC: CardioOncology state-of-the-art review. JACC CardioOncol. Published online July 3, 2025. doi:10.1016/j.jaccao.2025.05.010
2. Leiva O, Ren S, Neuberg D, et al. Pulmonary hypertension is associated with poor cardiovascular and hematologic outcomes in patients with myeloproliferative neoplasms and cardiovascular disease. Int J Hematol. 2023;117(1):90-99. doi:10.1007/s12185-022-03454-1
3. Leiva O, Alvarez-Cardona J, How J, Brunner A, Hobbs G. In-hospital and readmission outcomes of patients with myeloproliferative neoplasms and heart failure: Insights from the National Readmissions Database. Int J Cardiol Heart Vasc. 2023;49:101304. doi:10.1016/j.ijcha.2023.101304
4. Ferrari A, Scandura J, Masciulli A, Krichevsky S, Gavazzi A, Barbui T. Prevalence and risk factors for pulmonary hypertension associated with chronic myeloproliferative neoplasms. Eur J Haematol. 2021;106(2):250-259. doi:10.1111/ejh.13543
5. Leiva O, Soo S, Smilowitz N, et al. Myeloproliferative syndromes: clinical and epidemiological incidence, risk factors, and prognostic implications of heart failure hospitalizations among patients with myeloproliferative neoplasms. Blood. 2024;144(suppl 1):3188. doi:10.1182/blood-2024-207586
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