Prevalence and Effect on Survival of Pulmonary Hypertension in Myelofibrosis

Publication date: Available online 13 May 2019Source: Clinical Lymphoma Myeloma and LeukemiaAuthor(s): Matthew Austin, Peter J. Quesenberry, Corey E. Ventetuolo, Olin Liang, John L. ReaganAbstractMyelofibrosis (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. In this single center, retrospective chart review we identified 65 patients with biopsy proven primary and secondary myelofibrosis of whom 31 underwent transthoracic echocardiography. 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 echocardiographic evidence of Group 5 PH (Pulmonary Hypertension Due to Unclear or Multifactorial Mechanisms), 8 with primary myelofibrosis (PMF) and 6 with secondary MF. Myelofibrosis patients with PH trended toward being predominantly female, older, and less often had 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 PMF versus secondary MF and PMF intermediate risk patients. We conclude that given the high prevalence of MF associated PH, there may be a larger role for routine screening using echocardiography in MF patients. Further, the underlying association between PH and...
Source: Clinical Lymphoma Myeloma and Leukemia - Category: Cancer & Oncology Source Type: research

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