1. Pulmonary hypertension in patients with Philadelphia-negative myeloproliferative neoplasms: a single-center retrospective analysis of 225 patients
- Author
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Hyo Jin Lee, Jin-Ok Jeong, Yoon Seok Choi, Ik-Chan Song, Byung Joo Sun, Deog-Yeon Jo, Hwan Jung Yun, Myeong-Won Lee, and Hyewon Ryu
- Subjects
medicine.medical_specialty ,Myeloproliferative neoplasm ,Single Center ,Gastroenterology ,Essential thrombocytopenia ,Pulmonary hypertension ,03 medical and health sciences ,0302 clinical medicine ,Polycythemia vera ,Internal medicine ,medicine ,In patient ,Risk factor ,Myelofibrosis ,business.industry ,Essential thrombocythemia ,Hematology ,medicine.disease ,Primary myelofibrosis ,030220 oncology & carcinogenesis ,Original Article ,business ,030215 immunology - Abstract
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±284.2×109/L vs. 437.7±191.7×109/L, respectively; P =0.020) than PV patients without PH. PMF patients with PH had higher monocyte counts (1.3±0.5×109/L vs. 0.8±0.4×109/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.
- Published
- 2020