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Pulmonary Arterial Vasculopathy Associated with Graft Versus Host Disease following Hematopoietic Stem Cell Transplantation

K Brune

C. J. Tejada

K Miller

C Grossman

Daniel Grinnan


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Conference: 2012 International PHA Conference and Scientific Sessions

Release Date: 06.22.2012

Presentation Type: Abstracts

INTRODUCTION: Hematopoietic Stem Cell Transplantation (HSCT) has emerged as a viable therapeutic option for a variety of hematologic and solid malignancies. Pulmonary complications following HSCT, including graft versus host disease (GVHD), are common and often fatal. While many disorders of the pulmonary parenchyma have been described following HSCT, abnormalities of the pulmonary vascular system have been infrequently reported.

CASE REPORT: A 31 year old male received a matched, unrelated allogeneic HSCT for Philadelphia positive acute lymphoblastic leukemia in October 2006. Following transplantation, he developed chronic GVHD of the eyes, lungs, skin and gastrointestinal tract. He experienced progressive dyspnea with a decline in pulmonary function testing. Computed tomography of the chest revealed bronchiectasis and bilateral ground glass opacities consistent with interstitial lung disease. Bronchoscopy was performed and was negative for an infectious etiology. Despite treatment with immunosuppressive therapy and extracorporeal photopheresis, his dyspnea and radiographic abnormalities worsened. Open lung biopsy was performed, and his parenchymal disease was most consistent with lymphocytic interstitial pneumonitis, which has been described as a pathologic manifestation of pulmonary GVHD. Evaluation of his pulmonary vasculature revealed significant smooth muscle hypertrophy of the pulmonary arteries and arterioles in regions with and without associated parenchymal abnormalities.

DISCUSSION: While the focus of pulmonary complications following HSCT has been on the pulmonary parenchyma, we present a patient with significant pulmonary vasculopathy at the time of open lung biopsy. We propose that the pulmonary vascular system, in addition to the pulmonary parenchyma, may be affected in patients following HSCT, and we encourage further investigation of the pulmonary vasculature in this population.