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Diagnosis of pulmonary hypertension by noninvasive methods in hematopoietic cell transplant patients with myelofibrosis

Bone Marrow Transplantation volume 55, pages 1681–1683 (2020)Cite this article

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Pulmonary hypertension (PH) is a known complication of myelofibrosis (MF), although the underlying pathophysiology is poorly understood [1]. Physiologically, PH is defined by an elevated mean pulmonary arterial pressure >25 mmHg on right heart catheterization (RHC) [1]. PH can present as dyspnea and decreased exercise tolerance and if moderate or severe may lead to right-sided heart failure and death. The incidence of PH in patients with MF is unclear, and has been extrapolated from retrospective cohorts that include disparate chronic myeloproliferative neoplasms [2,3,4].

Allogeneic hematopoietic cell transplantation (HCT) is the only curative option for patients with MF. Patients with a score of Intermediate-2 or High risk by the Dynamic International Prognostic Scoring System (DIPSS) have a predicted life expectancy of <5 years and clearly derive a survival benefit from transplantation [5]. However, patients with the highest DIPSS scores also have the highest non-relapse mortality (NRM) after transplant [6]. PH is a known risk factor for cardiac death following allogeneic HCT, but the impact of pre-HCT PH on transplant outcomes in MF patients is unknown [7].

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Acknowledgements

The authors would like to thank Steve Liao who clarified the TTE interpretations. This research was funded in part through the NIH/NCI Cancer Center Support Grant P30 CA015704.

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Authors and Affiliations

  1. Clinical Research Division, Fred Hutchinson Cancer Research Center, Seattle, WA, USA

    Rachel B. Salit, Kelsey K. Baker, Greta Tobin, Ted A. Gooley, H. Joachim Deeg, Bart L. Scott & Guang-Shing Cheng

  2. Department of Medicine, University of Washington Medical Center, Seattle, WA, USA

    Rachel B. Salit, H. Joachim Deeg, Bart L. Scott & Guang-Shing Cheng

  3. Department of Radiology, University of Washington Medical Center, Seattle, WA, USA

    Rachael Edwards & Gregory Kicska

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Contributions

RBS and G-SC designed research, collected, and interpreted data and wrote the manuscript. RE determined the PA: Ao ratio on all CT scans. BLS and HJD critically revised the manuscript. GK collected data. KKB and TAG performed statistical analysis and contributed to the interpretation of the data.

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Correspondence to Rachel B. Salit.

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Salit, R.B., Baker, K.K., Edwards, R. et al. Diagnosis of pulmonary hypertension by noninvasive methods in hematopoietic cell transplant patients with myelofibrosis. Bone Marrow Transplant 55, 1681–1683 (2020). https://doi.org/10.1038/s41409-019-0769-9

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