title: Resolution of myelofibrosis-associated pulmonary arterial hypertension following allogeneic hematopoietic stem cell transplantation.
abstract: We present the case of a 62-year-old man with myelofibrosis-associated pulmonary arterial hypertension (PAH) who underwent allogeneic hematopoietic stem cell transplantation with subsequent resolution of disease and PAH. Right heart catheterization was used to guide PAH therapy before and after transplantation. Drug interactions, adverse effects, and renal insufficiency posed clinical challenges for the management of PAH-specific medications after transplantation. PAH improved soon after transplantation, and vasoactive medications were tapered off. Resolution of PAH was confirmed with repeat measurement of pulmonary hemodynamic characteristics. Although the etiology and pathophysiology for the resolution of PAH was unclear, the myelopulmonary pathophysiologic link was likely to have contributed. This is the first report describing resolution of myelofibrosis-associated PAH after allogeneic hematopoietic stem cell transplantation.
authors.affiliation: Department of Pulmonary Medicine, University of Texas MD Anderson Cancer Center, Houston, Texas, USA.
authors.forename: Saadia A
authors.initials: SA
authors.lastname: Faiz
authors.affiliation: Department of Cardiology, University of Texas MD Anderson Cancer Center, Houston, Texas, USA.
authors.forename: Cezar
authors.initials: C
authors.lastname: Iliescu
authors.affiliation: Department of Cardiology, University of Texas MD Anderson Cancer Center, Houston, Texas, USA.
authors.forename: Juan
authors.initials: J
authors.lastname: Lopez-Mattei
authors.affiliation: Division of Critical Care, Pulmonary and Sleep Medicine, University of Texas Health Science Center at Houston, Houston, Texas, USA.
authors.forename: Bela
authors.initials: B
authors.lastname: Patel
authors.affiliation: Department of Pulmonary Medicine, University of Texas MD Anderson Cancer Center, Houston, Texas, USA.
authors.forename: Lara
authors.initials: L
authors.lastname: Bashoura
authors.affiliation: Department of Stem Cell Transplantation, University of Texas MD Anderson Cancer Center, Houston, Texas, USA.
authors.forename: Uday
authors.initials: U
authors.lastname: Popat
keywords: cancer; hematopoietic stem cell transplantation; myelofibrosis; myeloproliferative disease; pulmonary arterial hypertension; pulmonary vascular disease