BCR-ABL1–Negative Chronic Myeloproliferative Neoplasms and Pulmonary Hypertension: A Prospective Long-Term Follow-up Study of the Impact of Pulmonary Hypertension on Survival

• Published in: Clinical lymphoma, myeloma and leukemia Vol. 21; no. 2; pp. 125 - 131
• Main Authors: Payzin, Kadriye Bahriye, Savasoglu, Kaan, Alacacioglu, Inci, Dalgic, Elif Ebru, Kucukzeybek, Betul Bolat, Calli, Aylin Orgen, Bener, Sadi, Payzin, Serdar
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01.02.2021
• Subjects: Adolescent; Adult; Aged; Aged, 80 and over; Echocardiography; Essential thrombocythemia; Female; Follow-Up Studies; Fusion Proteins, bcr-abl - analysis; Humans; JAK2 mutation; Janus Kinase 2 - genetics; Leukemia - complications; Leukemia - genetics; Leukemia - mortality; Male; Middle Aged; Mutation; Myeloproliferative Disorders - complications; Myeloproliferative Disorders - genetics; Myeloproliferative Disorders - mortality; Polycythemia vera; Prevalence; Primary myelofibrosis; Prospective Studies; Pulmonary Arterial Hypertension - diagnosis; Pulmonary Arterial Hypertension - epidemiology; Pulmonary Arterial Hypertension - genetics; Pulmonary Artery - diagnostic imaging; Pulmonary hypertension; Young Adult; Essential thrombocythemia; Polycythemia vera; JAK2 mutation; Primary myelofibrosis; Pulmonary hypertension
• ISSN: 2152-2650; 2152-2669
• DOI: 10.1016/j.clml.2020.08.006

To assess the prevalence of PHT in patients with BCR-ABL1–negative CMPN and to evaluate impact of PHT on survival during long-term follow-up. A total of 122 patients with BCR-ABL1–negative CMPN underwent transthoracic echocardiographic (TTE) evaluation at the beginning of study. Patients undergoing PHT on TTE examination were also evaluated by a pulmonologist. Patients were divided into 3 groups. Group A comprised patients with CMPN-related PHT; group B, patients with no PHT; and group C, patients with PHT due to secondary causes. Patients were evaluated again every 3 to 6 months. PHT was detected in 33 (27%) of 122 patients. Eight (6.5%) had CMPN-related PHT and the remaining 25 (20.5%) had non–CMPN-related PHT. Positivity for JAK2 V617F mutation in the study population was 72.9%. Groups were similar with respect to hematologic parameters and gender. Follow-up times were as follows: median (range) time from diagnosis to TTE and study end were 34 (1-158) months and 107 (16-251) months, respectively, and from TTE to study end was 88 (7-110) months. No significant differences found among the groups in terms of median time from diagnosis to TTE, follow-up, and overall survival. BCR-ABL1–negative CMPN patients had a lower prevalence of PHT compared to earlier studies. There was no statistically significant difference in median overall survival between patients with or without PHT. This may be because patients with PHT were asymptomatic and PHT was mild. The impact of PHT on survival was negligible. This prospective study assessed the prevalence of pulmonary hypertension (PHT) related to chronic myeloproliferative neoplasms (CMPNs) and evaluated the impact of PHT on survival during long-term follow-up. In a large group of BCR-ABL1–negative CMPN patients, there was a low prevalence of PHT. The impact of PHT on survival was negligible.