Reducing Cardiovascular Risk in Patients With Polycythemia Vera

Diagnosis and management of cardiovascular (CV) events including stroke, heart attacks, and peripheral arterial disease, may improve the outcomes of patients with myeloproliferative neoplasm (MPN) polycythemia vera (PV), who are at an increased risk of experiencing these events.

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Because high numbers of CV conditions and CV risk factors, such as obesity, hypertension, and diabetes are often observed in patients with MPNs, a multidisciplinary approach may need to be implemented when managing patients with PV. In this review, the researchers aimed to aid in understanding the importance of multidisciplinary management among this patient population.

“High blood pressure, smoking, and dyslipidemia are common in MPN and contribute to an increased risk of CV events,” wrote the researchers of the study. “Identifying patients at very high risk of fatal CV events is necessary to introduce early co-management by hematologists, cardiologists, and metabolic disease specialists.”

This review is published in Vascular Health and Risk Management.

PV is a rare disease characterized by aberrant myeloid lineage hematopoiesis with excessive red blood cell and pro-inflammatory cytokine production. Moreover, patients with PV often present with a range of thrombotic and hemorrhagic symptoms, which affect quality of life and are largely responsible for reduced overall survival expectancy and mortality. The current treatment landscape of PV is primarily focused on symptom control and improving survival through the prevention of thrombosis and leukemic transformation.

In this review, the researchers proposed to stratify CV risk in patients with PV, similar to what is recommended in the 2021 guidelines issued by the European Association of Preventative Cardiology/European Society of Cardiology (EAPC/ESC) systemic global cardiovascular disease (CVD) risk assessment in individuals with any major vascular risk.

Risk assessments are grouped as very-high, high, and moderate or low risk. Individuals with very high risk are referred to a cardiology or hypertension specialist, with high CV risk patients sometimes getting a referral. Moderate or low CV risk patients are directed to a general practitioner.

High CV risk individuals with modifiable risk factors, such as smoking, arterial hypertension, and dyslipidemia, were directed to either pharmaceutical management or lifestyle modifications.

The ESC/European Society of Hypertension (ESH) 2018 guidelines recommend patients with PV and arterial hypertension should be treated with angiotensin-converting enzyme inhibitors (ACE-1) or angiotensin II receptor antagonists (ARB) with or without calcium channel blockers to obtain a blood pressure reduction to the target of less than 130/80 millimeters of mercury (mmHg).

Similarly, ESC/European Atherosclerosis Society (EAS) recommend treatment of dyslipidemias with a target of 50% reduction low-density lipoprotein cholesterol (LDL-C) levels. However, lifestyle medications should be introduced as the first line of treatment, including diet, weight, and smoking factors.

Lastly, in the prevention of thrombotic events, recommendations include prior cytoreductive therapy and anticoagulant therapy for individuals with PV and prior thrombotic events. In the case of no prior thrombotic events, treatment recommendations include a hematocrit (Ht) target of less than 45%, acetylsalicylic acid (ASA) of 40-100 mg per day, and cytoreductive therapy for high-risk PV.

“The definition of CV risk class (percentage of fatal events at 10 years) based on the appropriate ESC scores is necessary to define the thresholds and intensity of intervention on pharmacologically modifiable risk factors, such as hypercholesterolemia, glucose metabolism, and blood pressure, and on risk factors modifiable with lifestyle, such as weight, diet, and smoking,” wrote the researchers. “Strict control of CV risk factors, in association with appropriate hematological therapy, may improve outcomes of patients with Ph-MPN [Ph-positive MPN].”

Reference

Benevolo G, Marchetti M, Melchio R, et al. Diagnosis and Management of Cardiovascular Risk in Patients with Polycythemia Vera. Vasc Health Risk Manag. 2023;19:765-778. Published 2023 Nov 22. doi:10.2147/VHRM.S429995