Characterization and prognostic implication of pulmonary hypertension among patients with myeloproliferative neoplasms
Pulmonary hypertension (PH) is a frequent complication of Philadelphia-negative myeloproliferative neoplasms (MPN), including essential thrombocythemia (ET), polycythemia vera (PV), and myelofibrosis (MF). However, its prognostic significance is understudied, thus we aimed to evaluate the effect of...
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Ferrata Storti Foundation
2025-05-01
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| Series: | Haematologica |
| Online Access: | https://haematologica.org/article/view/12061 |
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| author | Orly Leiva Steven Soo Olivia Liu Nathaniel R. Smilowitz Harmony Reynolds Binita Shah Samuel Bernard Joan How Michelle Hyunju Lee Gabriela Hobbs |
| author_facet | Orly Leiva Steven Soo Olivia Liu Nathaniel R. Smilowitz Harmony Reynolds Binita Shah Samuel Bernard Joan How Michelle Hyunju Lee Gabriela Hobbs |
| author_sort | Orly Leiva |
| collection | DOAJ |
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Pulmonary hypertension (PH) is a frequent complication of Philadelphia-negative myeloproliferative neoplasms (MPN), including essential thrombocythemia (ET), polycythemia vera (PV), and myelofibrosis (MF). However, its prognostic significance is understudied, thus we aimed to evaluate the effect of PH identified by echocardiography on risk of progression to secondary MF or acute leukemia in MPN patients. We conducted a multicenter, retrospective cohort study of MPN patients with ≥ 1 echocardiogram from 2010-2023. PH was defined as pulmonary artery systolic pressure (PASP) ≥ 40 mmHg. Outcomes were progression to secondary myelofibrosis or leukemia, major adverse cardiovascular event (MACE) and all-cause death. Multivariable Fine-Gray competing-risk regression was used to estimate subhazard ratio (SHR) of hematologic progression and MACE. 555 patients were included (42.7% PV, 41.1% ET, 16.2% MF) or which 195 (35.1%) had PH. Over a median follow-up period of 51.2 months, PH was associated with increased risk of secondary MF progression (aSHR 2.40, 95% CI 1.25–4.59), leukemia progression (aSHR 3.06, 95% CI 1.13 – 8.25), and MACE (aSHR 1.59, 95% CI 1.01– 2.49) but not all-cause death (aHR 1.48, 95% CI 0.96-2.26). Among patients with PH, absence of left heart disease (LHD) was associated with higher risk of secondary MF progression among patients with ET or PV (aSHR 2.76, 95% CI 1.19 – 6.38) and leukemia progression among patients with MF (aSHR 7.18, 95% CI 1.59-32.46). Prospective studies are needed to assess the role of echocardiography on MPNspecific prognostication.
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| format | Article |
| id | doaj-art-5b7de2403f034d44a0816f5b4c263053 |
| institution | OA Journals |
| issn | 0390-6078 1592-8721 |
| language | English |
| publishDate | 2025-05-01 |
| publisher | Ferrata Storti Foundation |
| record_format | Article |
| series | Haematologica |
| spelling | doaj-art-5b7de2403f034d44a0816f5b4c2630532025-08-20T01:51:42ZengFerrata Storti FoundationHaematologica0390-60781592-87212025-05-01999110.3324/haematol.2025.287497Characterization and prognostic implication of pulmonary hypertension among patients with myeloproliferative neoplasmsOrly Leiva0Steven Soo1Olivia Liu2Nathaniel R. Smilowitz3Harmony Reynolds4Binita Shah5Samuel Bernard6Joan How7Michelle Hyunju Lee8Gabriela Hobbs9Section of Cardiology - Heart Failure, Department of Medicine, University of Chicago, Chicago, IL; Division of Cardiology, Department of Medicine, New York University Grossman School of MedicineDepartment of Medicine, New York University Long Island School of Medicine, MineolaDivision of Cardiology, Department of Medicine, New York University Grossman School of MedicineDivision of Cardiology, Department of Medicine, New York University Grossman School of MedicineDivision of Cardiology, Department of Medicine, New York University Grossman School of MedicineDivision of Cardiology, Department of Medicine, New York University Grossman School of MedicineDivision of Cardiology, Department of Medicine, New York University Grossman School of MedicineDivision of Hematology and Oncology, Department of Medicine, Brigham and Women’s Hospital and Harvard Medical School, Boston, MADivision of Hematology and Oncology, Department of Medicine, Massachusetts General Hospital and Harvard Medical School, Boston, MADivision of Hematology and Oncology, Department of Medicine, Massachusetts General Hospital and Harvard Medical School, Boston, MA Pulmonary hypertension (PH) is a frequent complication of Philadelphia-negative myeloproliferative neoplasms (MPN), including essential thrombocythemia (ET), polycythemia vera (PV), and myelofibrosis (MF). However, its prognostic significance is understudied, thus we aimed to evaluate the effect of PH identified by echocardiography on risk of progression to secondary MF or acute leukemia in MPN patients. We conducted a multicenter, retrospective cohort study of MPN patients with ≥ 1 echocardiogram from 2010-2023. PH was defined as pulmonary artery systolic pressure (PASP) ≥ 40 mmHg. Outcomes were progression to secondary myelofibrosis or leukemia, major adverse cardiovascular event (MACE) and all-cause death. Multivariable Fine-Gray competing-risk regression was used to estimate subhazard ratio (SHR) of hematologic progression and MACE. 555 patients were included (42.7% PV, 41.1% ET, 16.2% MF) or which 195 (35.1%) had PH. Over a median follow-up period of 51.2 months, PH was associated with increased risk of secondary MF progression (aSHR 2.40, 95% CI 1.25–4.59), leukemia progression (aSHR 3.06, 95% CI 1.13 – 8.25), and MACE (aSHR 1.59, 95% CI 1.01– 2.49) but not all-cause death (aHR 1.48, 95% CI 0.96-2.26). Among patients with PH, absence of left heart disease (LHD) was associated with higher risk of secondary MF progression among patients with ET or PV (aSHR 2.76, 95% CI 1.19 – 6.38) and leukemia progression among patients with MF (aSHR 7.18, 95% CI 1.59-32.46). Prospective studies are needed to assess the role of echocardiography on MPNspecific prognostication. https://haematologica.org/article/view/12061 |
| spellingShingle | Orly Leiva Steven Soo Olivia Liu Nathaniel R. Smilowitz Harmony Reynolds Binita Shah Samuel Bernard Joan How Michelle Hyunju Lee Gabriela Hobbs Characterization and prognostic implication of pulmonary hypertension among patients with myeloproliferative neoplasms Haematologica |
| title | Characterization and prognostic implication of pulmonary hypertension among patients with myeloproliferative neoplasms |
| title_full | Characterization and prognostic implication of pulmonary hypertension among patients with myeloproliferative neoplasms |
| title_fullStr | Characterization and prognostic implication of pulmonary hypertension among patients with myeloproliferative neoplasms |
| title_full_unstemmed | Characterization and prognostic implication of pulmonary hypertension among patients with myeloproliferative neoplasms |
| title_short | Characterization and prognostic implication of pulmonary hypertension among patients with myeloproliferative neoplasms |
| title_sort | characterization and prognostic implication of pulmonary hypertension among patients with myeloproliferative neoplasms |
| url | https://haematologica.org/article/view/12061 |
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