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Pulmonary Vascular Disease |

Pulmonary Arterial Hypertension Associated With Graft-Versus-Host Disease After Hematopoietic Stem Cell Transplant

William Denney, MD; Kristin Miller, MD; Dan Grinnan, MD
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vcuhs, Richmond, VA


Chest. 2015;148(4_MeetingAbstracts):987A. doi:10.1378/chest.2271578
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Abstract

SESSION TITLE: Pulmonary Vascular Disease Student/Resident Case Report Posters

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM

INTRODUCTION: Pulmonary arterial hypertension (PAH) is a progressive disease of the pulmonary arterioles resulting in endothelial and smooth muscle proliferation. Cases of PAH after hematopoietic stem cell transplant (HSCT) and Graft-Versus-Host Disease (GVHD) have been described but are yet to have an established association. Here we present a case of PAH identified in an adult after allogenic HSCT and subsequent GVHD.

CASE PRESENTATION: A 49 year old female with B-cell acute lymphoblastic leukemia underwent allogenic HSCT in 03/2014. Her post-transplant course was complicated by cutaneous and gastrointestinal Grade II GVHD. Her GVHD was managed with tacrolimus, budesonide and triamcinolone. In 10/2014 she was admitted with sepsis and found to have a large right pleural effusion consistent with empyema and was treated with video assisted thorascopic surgery (VATS) with pleurodesis. During her recovery she was hypoxic and dyspneic. Transthoracic echocardiogram revealed normal left ventricular systolic function, dilated and hypertrophied right ventricle (RV) with reduced systolic function and severely elevated RV systolic pressure, 86 mmHg. She then underwent right heart catheterization (RHC). RHC showed pulmonary artery pressure (PAP) 75/30/45 mmHg, pulmonary capillary wedge pressure (PCWP) 7 mmHg, pulmonary vascular resistance (PVR) 10.3 Woods Units and cardiac output (CO) 3.68 L/min. She responded to inhaled nitrous oxide, with PAP 60/22/35 mmHg, PCWP 8 mmHg, PVR 6.2 Woods Units and CO 4.37 L/min. Alternate etiologies of PH were assessed and led to a diagnosis of PAH, and she was started on sildenafil.

DISCUSSION: While PH has been associated with GVHD following HSCT in children [1], it has rarely been diagnosed in adults with GVHD [2]. In this case report, we present an adult who developed PAH in association with GVHD following HSCT. As GVHD is known to increase endothelial injury and induce cytokines implicated in the development of PAH [3], an association between GVHD and PAH is important to note.

CONCLUSIONS: While PH has been associated with GVHD following HSCT in children [1], it has rarely been diagnosed in adults with GVHD [2]. In this case report, we present an adult who developed PAH in association with GVHD following HSCT. As GVHD is known to increase endothelial injury and induce cytokines implicated in the development of PAH [3], an association between GVHD and PAH is important to note.

Reference #1: Dandoy, C. E., Hirsch, R., Chima, R., Davies, S. M., & Jodele, S. Pulmonary hypertension after hematopoietic stem cell transplantation. Biology of Blood and Marrow Transplantation 2013; 19(11), 1546-1556.

Reference #2: Grigg A, Buchanan M, Whitford H. Late-onset pulmonary arterial hypertension in association with graft-versus-host disease after allogeneic stem-cell transplantation. Am J Hematol 2005;80:38-42.

Reference #3: Daly AS, Xenocostas A, Lipton JH. Transplantation-associated thrombotic microangiopathy: twenty-two years later. Bone Marrow Transplant 2002; 30: 709-715.

DISCLOSURE: The following authors have nothing to disclose: William Denney, Kristin Miller, Dan Grinnan

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