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Master Thesis

L’hypertension pulmonaire chez les patients avec un syndrome myéloprolifératif

Abstract : Introduction: Chronic myeloproliferative neoplasms (MPN) are recognized as a cause of pulmonary hypertension (PH) in the World Health Organization (WHO) 2016 classification of myeloid neoplasm. Methods: From January 2008 to May 2018, 183 newly consecutive MPN patients from the Hematology and Cellular Therapy Department of University Hospital of Marseille (France) have been evaluated by transthoracic echocardiography (TTE) to detect pulmonary hypertension (PH). Diagnosis of chronic eosinophilic leukemia included 2 patients (1%), 4 (2%) patients had secondary myelofibrosis (SMF), 11 (6%) patients had primary myelofibrosis (PMF), 28 (15%) patients had chronic myeloid leukemia (CML), 51 (28%) patients had polycythemia vera (PV) and 87 patients (47%) had essential thrombocythemia (ET). Results: Based on guidelines of the European Society of Cardiology PH was suspected by TTE in 16 patients (8.8%) among our 183 evaluated patients: 4 with PV (25%), 7 with ET (43.8%), 2 with PMF (12.5%) and 3 with CML (18.8%). Because of a global cardiac failure 2 patients with ET were excluded. The remaining 14 patients had no left-sided cardiac or lung disease. Three patients underwent a right heart catheterization (RHC) with confirmation of the PH. Two patients with PH were dead at the last check-point of our study (May 2018), one with ET and one with PMF. The median systolic pulmonary artery pressure (sPAP) for the fourteen patients with PH was 46.5 mmHg (range: 35–85). Conclusion In this large cohort of 183 MPN patients evaluated by TTE to diagnose PH, 16 patients (8.8%) developed a PH. Among these 16 PH patients, 2 had global cardiac failure at diagnosis time whereas the remaining 14 (7.7%) patients had neither cardiac or lung disease and no history of thromboembolic disease, directly involving MPN in PH development. However, this prevalence is lower than expected regarding the data previously reported.
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https://dumas.ccsd.cnrs.fr/dumas-02010320
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Submitted on : Thursday, February 7, 2019 - 9:59:36 AM
Last modification on : Wednesday, July 15, 2020 - 2:21:40 PM
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Mihail Turcanu. L’hypertension pulmonaire chez les patients avec un syndrome myéloprolifératif. Sciences du Vivant [q-bio]. 2018. ⟨dumas-02010320⟩

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