SESSION TITLE: Transplant Cases
SESSION TYPE: Affiliate Case Report Slide
PRESENTED ON: Sunday, October 26, 2014 at 03:15 PM - 04:15 PM
INTRODUCTION: Bronchiolitis obliterans (BO) is a manifestation of graft versus host disease (GVHD) in allogenic bone marrow transplant (BMT) patients. Herein we report an extreme manifestation of pulmonary GVHD.
CASE PRESENTATION: The patient is a 25-year old male with a medical history significant for severe aplastic anemia status-post allogenic matched sibling BMT. Patient was conditioned with cyclophosphamide and Anti-Thymocyte Globulin with total body irradiation of 2 Gy. Patient developed refractory GVHD (grade 4), which improved with high-dose steroids in addition to tacrolimus. Two years after transplant, dyspnea developed and a CT chest showed new mosaic pattern of groundglass opacities. Pulmonary function tests were significant for new obstruction with airtrapping. Transbronchial biopsy was consistent with GVHD. He was initiated on fluticasone, montelukast, azithromycin and prednisone therapy with initial improvement. One year later, he presented with chest pain and dyspnea. CT chest noted right pneumothorax and severe bullous changes. A chest tube was placed. Due to persistent air leak, he underwent a right video-assisted thoracoscopy (VATS) with bleb resection. One year later, he underwent left VATS with bleb resection for recurrent pneumothorax. Despite surgical intervention, he continued to experience recurrent pneumothoraces and spontaneous pneumomediastinum. He is currently hypoxemic with very severe obstruction completing pulmonary rehabilitation. Unfortunately, he was not a candidate for lung transplant due to multiple risk factors.
DISCUSSION: BO is suggestive of chronic GVHD with a median onset after BMT of 18 months and a 5-year mortality of 41%.1 Treatment includes glucocorticoids and increased immunosuppression. Azithromycin, montelukast, and extracorporeal photopheresis have been found to slow rate of decline in pulmonary function. Lung transplantation may be beneficial. Early symptoms are mild dyspnea and dry cough, which later develops into reduced exercise tolerance. There are few case reports of bilateral pneumothoraces and pneumomediastinum both with and without bullae as a complication of BO.
CONCLUSIONS: We report a rare consequence of BO after BMT with severe bullous changes, spontaneous pneumomediastinum, recurrent pneumothoraces, and need for bilateral bullectomy which was ineffective.
Reference #1: Ditschkowski M, Elmaagacli AH, Koldehoff M, et al. Bronchiolitis obliterans after allogeneic hematopoietic SCT: further insight--new perspectives? Bone marrow transplantation 2013; 48:1224-1229
DISCLOSURE: The following authors have nothing to disclose: Diana Kelm, Mark Litzow, Cassie Kennedy
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