A recent study published in Haematologica indicates that pulmonary hypertension (PH) in individuals diagnosed with myelofibrosis (MF) correlates with a heightened likelihood of hematologic advancement and adverse cardiovascular incidents.

According to 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,”…[PH] has been associated with myeloproliferative neoplasms (MPNs),although its implications on prognosis have not been well characterized.” They told MedPage Today that “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.”

The US-based, retrospective, multicenter cohort study sought to assess PH’s impact on the progression risk to secondary MF or acute leukemia in MPN patients. the study involved 555 participants, aged 18 and above, with MPNs, including essential thrombocythemia (ET; 41.1%), polycythemia vera (PV; 42.7%), or MF (16.2%), all of whom underwent at least one TTE post-initial diagnosis. The primary focus was the advancement to secondary MF and acute leukemia, while secondary concerns included major adverse cardiovascular events (MACE), encompassing arterial or venous thrombosis, heart failure hospitalization, or cardiovascular death, alongside overall mortality.

Within the patient group, 35.1% received a PH diagnosis, defined by a pulmonary artery systolic pressure (PASP) of ≥ 40 mmHg. Over a median follow-up duration of 51.2 months, PH showed a significant link to increased risks of disease progression, notably 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). Though, PH did not exhibit a significant correlation with all-cause mortality.

Further analysis based on MPN subtypes revealed similar patterns among ET or PV patients, where PH also linked to a greater risk of MF progression and leukemia transformation. Conversely, in patients with established MF, PH did not seem to influence MACE rates, leukemia progression, or overall mortality.

The researchers also explored the role of left heart disease (LHD) in PH patients. Those without LHD faced a considerably higher risk of secondary MF progression compared to those with LHD (21.3% vs 10.5%; P =.027), alongside a decreased MACE risk (41.5% vs 57.6%; P = .015). Multivariable analysis corroborated these results, indicating that the absence of LHD might pinpoint a subgroup at heightened risk for hematologic advancement, particularly among individuals with ET, PV, or MF.

These results underscore PH’s prognostic importance as a marker for MPN disease progression. Regular cardiovascular evaluations, including echocardiography, could aid in identifying high-risk patients, facilitating more customized monitoring and treatment approaches.

according to hobbs, “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,” as reported in medpage Today.

Study Highlights Prognostic Significance of Pulmonary Hypertension

“Our results suggest that clinicians shouldn’t ignore PH on echocardiography…and that close attention to cardiovascular and hematologic progression is needed.”

The research emphasizes the importance of monitoring pulmonary hypertension in patients with myeloproliferative neoplasms to better manage disease progression and cardiovascular risks.