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Pulmonary hypertension is associated with increased nonrelapse mortality after allogeneic hematopoietic cell transplantation for myelofibrosis

Bone Marrow Transplantation volume 55, pages 877–883 (2020)Cite this article

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Abstract

Allogeneic hematopoietic cell transplantation (alloHCT) is the only curative therapy for primary myelofibrosis (MF) as well as myelofibrosis secondary to other myeloproliferative neoplasms (MPN). Pulmonary hypertension (PH) is a known complication of MF and may occur in up to 50% of such patients. PH (defined as a mean pulmonary artery pressure ≥25 mmHg at rest) can eventually lead to right heart failure and may be associated with complications after alloHCT. We examined the association of PH with alloHCT outcome in patients with MF associated with MPN. Pre- and post-HCT echocardiograms were reviewed to estimate the peak pulmonary artery systolic pressure (PASP). Median PASP was 37.0 mmHg (range: 16.0-57.9) prior to HCT with 37 of 65 patients (57%) studied. With median follow-up of 35.0 months (range: 3.3–119.4) PH was significantly associated with inferior OS (58.9% vs. 88.8%, P = 0.025), primarily due to increased NRM (21.6% vs. 7.1%, P = 0.007). The majority of the deaths (8 of 14) in patients with PH occurred within 100 days after HCT. In patients with an available post-HCT echocardiogram (n = 33), the median PASP was 30 mmHg (range: 5.0–56.2); eight patients (24%) had persistent PH. Compared with pre-HCT values, PASP was significantly reduced after HCT (p < 0.001). We conclude that PH is associated with inferior survival due to the increased NRM in patients with MF undergoing alloHCT. PH appears at least partially reversible after successful alloHCT. PH should be considered a risk factor for early mortality after alloHCT and surveillance of pulmonary artery pressure in MF patients being considered for alloHCT may be useful.

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Authors and Affiliations

  1. Department of Hematology and Hematopoietic Cell Transplantation, City of Hope Medical Center, Duarte, CA, USA

    Rohan Gupta, Faizi Jamal, Ibrahim Aldoss, Monzr Al Malki, Matthew Mei, Amandeep Salhotra, Surime Dobrin, Anthony S. Stein, Karamjeet Sandhu, Samer Khaled, Ahmed Aribi, Guido Marcucci, Stephen J. Forman, David Snyder, Ryotaro Nakamura, Haris Ali & Vinod Pullarkat

  2. Department of Information Sciences, City of Hope Medical Center, Duarte, CA, USA

    Dongyun Yang & Joycelynne Palmer

  3. Department of Cardiology, City of Hope Medical Center, Duarte, CA, USA

    Chendri Chendri, Michael Tran & K. Venkataraman

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  1. Rohan Gupta
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Correspondence to Vinod Pullarkat.

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Gupta, R., Jamal, F., Yang, D. et al. Pulmonary hypertension is associated with increased nonrelapse mortality after allogeneic hematopoietic cell transplantation for myelofibrosis. Bone Marrow Transplant 55, 877–883 (2020). https://doi.org/10.1038/s41409-019-0741-8

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