Geoffroy Venton1, Mihai Turcanu2, Julien Colle1, Franck Thuny3, Safia Chebrek4, Laure Farnault5, Cédric Mercier6, Vadim Ivanov5, Raphaëlle Fanciullino7, Pierre Suchon8, Pierre-André Jarrot9, Karim Aissi10, Pauline Roche5, Jennifer Cautela3, Robin Arcani11, Regis Costello1. 1. Hematology and Cellular Therapy Department, La Conception, University Hospital of Marseille, France; Aix-Marseille University, INSERM, UMR1090 TAGC, Marseille F_13288, France. 2. Hematology and Cellular Therapy Department, La Conception, University Hospital of Marseille, France; General Medicine Department, Aix-Marseille University, France. 3. Aix-Marseille University, Assistance Publique - Hopitaux de Marseille, Mediterranean University Cardio-Oncology Center, Unit of Heart Failure and Valvular Heart Diseases, Department of Cardiology, Hôpital Nord, Marseille, France; Aix-Marseille University, Inserm, Inra, Centre for Cardio-Vascular and Nutrition research (C2VN), Marseille, France. 4. Hematology Department, Hospital of Avignon, Marseille, France. 5. Hematology and Cellular Therapy Department, La Conception, University Hospital of Marseille, France. 6. Hematology and Cellular Therapy Department, La Conception, University Hospital of Marseille, France; UMR-911 INSERM, Toxicokinetics and Pharmacokinetics Laboratory, Pharmacological Faculty, Marseille, France. 7. SMARTc Unit, Pharmacokinetics Laboratory, UMR_911 CRO2 AMU, Marseille, France; Pharmacy Unit, La Conception, University Hospital of Marseille, APHM, Marseille, France. 8. Hematology Laboratory, La Timone, University Hospital of Marseille, France; UMR 1062 NORT, INSERM, Marseille, France. 9. Internal Medicine and Clinic Immunology Department, La Conception, University Hospital of Marseille, France; Center for Cardiovascular and Nutrition Research, INRA 1260, INSERM _S1263, Aix-Marseille University, France. 10. Internal Medicine Department, North Hospital, University Hospital of Marseille, France. 11. Hematology and Cellular Therapy Department, La Conception, University Hospital of Marseille, France. Electronic address: robin.arcani@ap-hm.fr.
Abstract
BACKGROUND: Chronic myeloproliferative neoplasms (MPN) are recognized as a cause of pulmonary hypertension (pH). We ought to describe the prevalence and characteristics of PH in a cohort of MPN who were screened using transthoracic echocardiography (TTE). METHODS: One hundred eighty-three newly diagnosed consecutive MPN patients were prospectively evaluated using TTE to detect PH. RESULTS: Two patients were diagnosed with chronic eosinophilic leukemia, two patients had post-essential thrombocythemia (ET) myelofibrosis (MF), two patients had post-polycythemia vera (PV) MF, 11 patients had primary myelofibrosis (PMF), 28 patients had chronic myeloid leukemia (CML), 51 patients had PV, and 87 patients had ET. TTE was used to determine PH, and PH was suspected in 16 of 183 patients as follows: four with PV, seven with ET, two with PMF, and three with CML. Two patients with ET were excluded because of global cardiac failure. Three patients underwent right heart catheterization to confirm PH. The 14 (7.7%) patients with PH had no cardiac or lung disease that directly involved MPN in PH development. CONCLUSION: In this large cohort of 183 MPN patients, TTE was used to diagnose PH, and 14 patients (7.7%) developed PH. This prevalence was lower than expected based on previously reported data, but it remains higher than in the general population.
BACKGROUND:Chronic myeloproliferative neoplasms (MPN) are recognized as a cause of pulmonary hypertension (pH). We ought to describe the prevalence and characteristics of PH in a cohort of MPN who were screened using transthoracic echocardiography (TTE). METHODS: One hundred eighty-three newly diagnosed consecutive MPN patients were prospectively evaluated using TTE to detect PH. RESULTS: Two patients were diagnosed with chronic eosinophilic leukemia, two patients had post-essential thrombocythemia (ET) myelofibrosis (MF), two patients had post-polycythemia vera (PV) MF, 11 patients had primary myelofibrosis (PMF), 28 patients had chronic myeloid leukemia (CML), 51 patients had PV, and 87 patients had ET. TTE was used to determine PH, and PH was suspected in 16 of 183 patients as follows: four with PV, seven with ET, two with PMF, and three with CML. Two patients with ET were excluded because of global cardiac failure. Three patients underwent right heart catheterization to confirm PH. The 14 (7.7%) patients with PH had no cardiac or lung disease that directly involved MPN in PH development. CONCLUSION: In this large cohort of 183 MPN patients, TTE was used to diagnose PH, and 14 patients (7.7%) developed PH. This prevalence was lower than expected based on previously reported data, but it remains higher than in the general population.