Pulmonary Hypertension Associated With Increased Risk of Progression in Patients With Myeloproliferative Neoplasms

Pulmonary hypertension (PH) in patients with myelofibrosis (MF) is associated with increased risk of hematologic progression, adverse cardiovascular events, and all-cause mortality, according to study findings published in Haematologica.¹

Visualization of pulmonary hypertension | Image Credit: © Thirawat - stock.adobe.com

“…[PH] has been associated with myeloproliferative neoplasms (MPNs), although its implications on prognosis have not been well characterized,” Gabriela Hobbs, MD, a hematologist/oncologist at Massachusetts General Hospital and Harvard Medical School, and Orly Leiva, MD, a cardiologist at the University of Chicago, told MedPage Today. “Transthoracic echocardiography (TTE) is a noninvasive test that’s relatively inexpensive and widely available in the United States and is used for the screening of PH by noninvasively estimating pulmonary artery systolic pressure.”²

In a US-based, retrospective, multicenter cohort study, researchers aimed to evaluate the effect of PH on the risk of progression to secondary MF or acute leukemia in patients with MPN. They assessed a total of 555 patients ages 18 years and older with MPNs such as essential thrombocythemia (ET; 41.1%), polycythemia vera (PV; 42.7%), or MF (16.2%) who had 1 or more TTE after their initial diagnosis. The primary end points were progression to secondary MF and acute leukemia. Secondary end points included major adverse cardiovascular events (MACE)—a composite measure of arterial or venous thrombosis, heart failure hospitalization, or cardiovascular death—as well as all-cause mortality.^1,2

Of the patient population, 35.1% were diagnosed with PH (defined as pulmonary artery systolic pressure (PASP) ≥ 40 mmHg). At a median follow-up of 51.2 months, PH was significantly associated with an increased risk of disease progression events, including secondary MF (SHR 2.40, 95% CI 1.25–4.59), leukemia transformation (SHR 3.06, 95% CI 1.13–8.25), and MACE (SHR 1.59, 95% CI 1.01–2.49). Notably, PH was not significantly linked to all-cause mortality.^1,2

Further stratification by MPN subtype showed similar trends among patients with ET or PV, where PH was also associated with increased risk of MF progression and leukemia transformation. However, among patients with established MF, PH did not appear to impact rates of MACE, leukemia progression, or all-cause death.^1,2

Investigators also examined the influence of left heart disease (LHD) among patients with PH. Those without LHD had a significantly higher risk of secondary MF progression compared with those with LHD (21.3% vs 10.5%; P = .027), as well as a reduced risk of MACE (41.5% vs 57.6%; P = .015). Multivariable analysis confirmed these findings, suggesting that the absence of LHD may identify a subgroup at elevated risk for hematologic progression, particularly among patients with ET, PV, or MF.^1,2

These findings highlight the prognostic significance of PH as a marker for disease progression in MPNs. Incorporating routine cardiovascular assessment, including echocardiography, may help identify high-risk patients and guide more tailored monitoring and therapeutic strategies.

"Our results suggest that clinicians shouldn’t ignore PH on echocardiography...and that close attention to cardiovascular and hematologic progression is needed among patients with [MPNs] and PH,” Hobbs said in MedPage Today.²

REFERENCES

1. Leiva O, Soo S, Liu O, et al. Characterization and prognostic implication of pulmonary hypertension among patients with myeloproliferative neoplasms. Haematologica. May 15, 2025. Accessed June 30, 2025. Doi:10.3324/haematol.2025.287497

2. In patients with myeloproliferative neoplasms, this discovery could signal trouble. MedPage Today. June 27, 2025. Accessed June 30, 2025. https://www.medpagetoday.com/resource-centers/myelofibrosis-focus/patients-myeloproliferative-neoplasms-discovery-could-signal-trouble/5834