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

Abstract Aim In this prospective study, we aimed 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. Methods A total of 122 patients with BCR-ABL1 negative CMPN underwent transthoracic echocardiographic (TTE) evaluation at the beginning of study. Patients having PHT on TTE examination, were also evaluated by a pulmonologist. Patients were analyzed in three groups. Group A: Patients with CMPN related PHT, Group B: Patients with no PHT, and Group C: Patients with PHT due to secondary causes. Patients re-evaluated every 3-6 months. Results PHT was detected in 33 of 122 (27%) patients. Eight of them (6.5%) had CMPN related PHT and remaining 25 (20.5%) had non-CMPN related PHT. Positivity for JAK2 V617F mutation in study population was 72.9%. Groups were similar with respect to hematologic parameters and gender. Follow-up times were as follows: From diagnosis to TTE and study end were 34 (1-158), 107 (16-251) months respectively and from TTE to study end 88 (7-110) months. No significant differences found among the groups in terms of median time from diagnosis to TTE, follow-up, and the OS Conclusion In BCR-ABL1 negative CMPN patients we found lower prevalence of PHT when compared to earlier studies. There was no statistically significant difference on median overall survival between the patients with or without PHT. This may be because of patients with PHT were asymptomatic and, PHT was mild. Impact of PHT on survival was negligible at this point.