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Prevalence of pulmonary hypertension in myelofibrosis

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Pulmonary hypertension (PH) has been described in myelofibrosis (MF), but it is rare and typically found in advanced disease. Although the etiology of PH in MF is unclear, early predictors may be detected by echocardiogram. The goals of our study were to evaluate the prevalence of PH as determined by echocardiography in a cohort of MF patients and to identify clinical risk factors for PH. We performed a retrospective review of MF patients from October 2015 to May 2017 at MD Anderson Cancer Center in the ambulatory clinic, and those with echocardiogram were included. Clinical, echocardiographic, and laboratory data were reviewed. Patients with and without PH were compared using a chi-square or Fisher’s exact test, and logistic regression was performed with an outcome variable of PH. There were 143 patients with MF who underwent echocardiogram, and 20 (14%) had echocardiographic findings consistent with PH. Older age, male gender, hypertension, hyperlipidemia, coronary artery disease, dyspnea, hematocrit, brain natriuretic peptide (BNP), and N-terminal prohormone BNP (NT-proBNP) were significantly different between those without PH and those with PH (p < 0.05). Female gender was protective (OR 0.21, 95% CI 0.049–0.90, p = 0.035), and NT-proBNP was a significant clinical predictor of PH (OR 1.07, CI 1.02 = 1.12, p = 0.006). PH in MF is lower than previously reported in our MF cohort, but many patients had cardiac comorbidities. PH due to left-sided heart disease may be underestimated in MF. Evaluation of respiratory symptoms and elevated NT-proBNP should prompt a baseline echocardiogram. Early detection of PH with a multidisciplinary approach may allow treatment of reversible etiologies.

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We thank Dr. George A. Eapen for his assistance in the editorial review of this article.


This research is supported in part by the National Institutes of Health through MD Anderson Cancer Center Support Grant (CA016672).

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JL, SV, SF had full access to the all of the data in the study and takes responsibility for the integrity of the data and accuracy of the data analysis, including and especially any adverse effects. JL, SV, KB, BG, HG contributed substantially to the study design, data collection, data analysis and interpretation, and the writing of the manuscript. BF, CI, SH, PK, NP, and MM contributed to data analysis and interpretation, review and editing of the manuscript.

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Correspondence to Saadia A. Faiz.

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Lopez-Mattei, J., Verstovsek, S., Fellman, B. et al. Prevalence of pulmonary hypertension in myelofibrosis. Ann Hematol 99, 781–789 (2020).

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