Prevalence and Effect on Survival of Pulmonary Hypertension in Myelofibrosis

Myelofibrosis (MF), a rare disorder characterized by bone marrow fibrosis, has been implicated as a cause of pulmonary hypertension (PH). To date, studies examining this association have not looked at the impact of PH on survival in MF. We examined the relationship between MF and PH by echocardiogram (echo) using a retrospective patient database and examined the influence of PH on overall survival.

In this single-center retrospective chart review, we identified 65 patients with biopsy-proven primary and secondary MF, 31 of whom underwent transthoracic echo. After accounting for chronic obstructive pulmonary disease and left-sided or valvular heart dysfunction, which excluded 6 patients, we identified 14 patients (56%) who had echo evidence of group 5 PH (ie, PH due to unclear or multifactorial mechanisms), 8 with primary MF and 6 with secondary MF. MF patients with PH trended toward being predominantly female, being older, and less often having constitutional symptoms compared to the non-PH cohort.

There was no effect of the presence of PH on overall survival in the entire MF cohort or in any subgroup analyzed, including primary MF versus secondary MF and primary MF intermediate risk patients.

Given the high prevalence of MF-associated PH, there may be a larger role for routine echo screening in MF patients. Further, the underlying association between PH and MF may signify an endothelial plasticity or increased telomerase activity as part of the pathogenesis of MF.