Prevalence of Pulmonary Hypertension in Patients with Myeloproliferative Neoplasms

By Kerri Fitzgerald - Last Updated: February 11, 2021

Pulmonary hypertension is common in patients with Philadelphia?chromosome negative myeloproliferative neoplasms (MPN). Pulmonary hypertension can be diagnosed by right heart catheterization (RHC) or estimated by transthoracic echocardiography (TTE). Pulmonary hypertension impacts survival in this patient population, but estimates of its prevalence varies and risk factors are poorly established. A study published in the European Journal of Haematology found that about one-third of patients with MPN experience pulmonary hypertension.

Researchers conducted a systematic review and meta?analysis of EMBASE, Medline, and to identify studies from 1999 to 2019 involving pulmonary hypertension, MPN, polycythemia vera (PV), essential thrombocytopenia, and/or myelofibrosis (MF). A total of 17 papers met the study’s inclusion criteria, comprising 935 patients.

Among the patient cohort analyzed, 309 (33%) had pulmonary hypertension. Overall, the prevalence of pulmonary hypertension varied greatly across studies from 3.8% to 58.0%.

Via logistic mixed?effect regression, researchers found that diagnosis mode (RHC vs. TTE) impacted the prevalence of pulmonary hypertension. Compared with TTE, the odds ratio (OR) for pulmonary hypertension diagnosis with RHC was 0.1 (95% confidence interval [CI], 0.01-0.9). Studies that predominantly used TTE yielded prevalence estimates approximately fivefold higher than those using RHC (35% vs. 7.2%).

Age and duration of MPN disease were significant risk factors for pulmonary hypertension. Compared with a diagnosis of PV, a diagnosis of MF had a more than twofold association with risk of pulmonary hypertension (OR, 2.6; 95% CI, 1.36-4.8). Primary MF and secondary MF were both more highly associated with pulmonary hypertension than PV.

“Patients with MF and longer duration of [MPN] disease seem at particularly high risk and should be carefully monitored for pulmonary hypertension,” the researchers concluded.