Pulmonary hypertension in patients with Philadelphia-negative myeloproliferative neoplasms: a single-center retrospective analysis of 225 patients

BACKGROUND: The prevalence of pulmonary hypertension (PH) in myeloproliferative neoplasms (MPNs) varies among studies. We analyzed the prevalence of PH in Korean patients with Philadelphia-negative (Ph(-)) MPNs. METHODS: Medical records of patients with Ph(-) MPNs [essential thrombocythemia (ET), polycythemia vera (PV), or primary myelofibrosis (PMF)] visiting a single hospital between 1993 and 2019 were reviewed retrospectively. Transthoracic echocardiographic examination (TTE) results were reviewed and PH was diagnosed according to established guidelines. RESULTS: Of the 320 MPN (179 ET, 107 PV, and 34 PMF) patients, 225 (121 ET, 83 PV, and 21 PMF) underwent TTE. Of these 225 MPN patients, 19 of 121 (15.7%) ET, 9 of 83 (10.8%) PV, and 6 of 21 (28.6%) PMF patients had PH. PV patients with PH were older [71 (42‒85) vs. 61.5 (26‒91) yr, respectively; P=0.049], predominantly female (male:female ratio, 0.29 vs. 1.96, respectively; P=0.010), had lower hemoglobin levels (15.9±2.6 g/dL vs. 18.4±2.6 g/dL, respectively; P=0.010), and higher platelet counts (616.6±284.2×10(9)/L vs. 437.7±191.7×10(9)/L, respectively; P=0.020) than PV patients without PH. PMF patients with PH had higher monocyte counts (1.3±0.5×10(9)/L vs. 0.8±0.4×10(9)/L, respectively; P=0.031) than those without PH. PH was a risk factor for poor survival in PV (HR, 12.4; 95% CI, 1.8‒86.6). CONCLUSION: PH is common in patients with Ph(-) MPNs and hence, careful screening for PH is warranted.