MULTIPLE CHEST CT FINDINGS IN A POST-TRANSPLANT PATIENT

Abstract

SESSION TITLE: Fellows Pulmonary Manifestations of Systemic Disease Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: October 18-21, 2020 INTRODUCTION: Pulmonary complications are common in hematopoietic stem cell transplantation recipients. Late complications occur more than 100 days after HSCT. During this time frame noninfectious causes are more common than infectious complications. CASE PRESENTATION: 57 y/o Panamanian female never-smoker with history of Common Variable Immune Deficiency, and recurrent non-Hodgkin lymphoma status post-chemotherapy and autologous stem cell transplant 2015 followed by allogeneic bone marrow transplant in 2017 presented with dyspnea and non-productive cough. She had presumed pneumonia two months prior but denied infectious or constitutional symptoms. Initial CT chest demonstrated diffuse bilateral GGOs and PFTs had a restrictive pattern with decreased DLCO. PET CT lacked findings suggesting recurrent lymphoma and connective tissue disease. Initial differential diagnosis included infection, organizing pneumonia, and eosinophilic lung disease. The patient underwent bronchoscopy with bronchoalveolar lavage (negative cultures and cytology). Prednisone therapy for presumed cryptogenic organizing pneumonia was initiated. Shortly after she developed worsening dyspnea, tachycardia and new exertional hypoxia and was found to have pneumomediastinum. Bronchoscopy was repeated and again cultures and cytology negative and TTE demonstrated only mild diastolic dysfunction. Without an alternative diagnosis treatment for PCP was provided for 21 days and 50 mg prednisone continued. At completion of treatment there was no improvement on her CT. A surgical lung biopsy was performed, and multidisciplinary radiologic-pathologic diagnosis of pleuroparenchymal fibroelastosis (PPFE) was rendered. DISCUSSION: Predominant findings such as the GGO and pneumomediastinum, can overshadow less conspicuous features such as PPFE which can be confounding. PPFE is a rare disorder usually occurring between age 40 to 70. Numerous potential risk factors for the development of PPFE have been described most common of which are bone marrow, hematopoietic stem cell and lung transplant along with CTD, familial, post infectious, and idiopathic. Clinical features include exertional dyspnea, insidious dry cough, and weight loss. Typically a restrictive ventilatory defect and reduced DLCO are noted. In 2012 Reddy and colleagues proposed diagnostic criteria integrating histopathologic and radiographic features included bilateral upper zone pleural thickening with associated subpleural fibrosis on CT and intraalveolar fibrosis and elastosis on histopathological exam. Pneumothorax and pneumomediastinum are frequent complications of PPFE. CONCLUSIONS: PPFE is a rare condition but should be considered in patients a history of transplant. Treatment remains a clinical challenge and lung transplant is an option. In our patient, possible contributing factors for the development of PPFE were recurrent pulmonary infection and late-onset complication following HSCT. Reference #1: Pena E, Souza CA, Escuissato DL, et al. Noninfectious pulmonary complications after hematopoietic stem cell transplantation: practical approach to imaging diagnosis. Radiographics 2014; 34: 663–683 Reference #2: Chua, F, Desai, SR, Nicholson, AG, et al. Pleuroparenchymal fibroelastosis: a review of clinical, radiological and pathological characteristics. Ann Am Thorac Soc 2019; 16: 1351–1359 Reference #3: Reddy TL, Tominaga M, Hansell DM, von der Thusen J Jr, Rassl D, Parfrey H, Guy S, Twentyman O, Rice A, Maher TM, et al. Pleuroparenchymal fibroelastosis: a spectrum of histopathological and imaging phenotypes. Eur Respir J 2012;40:377–385 DISCLOSURES: No relevant relationships by Sally Delvecchio, source=Web Response No relevant relationships by Teri Franks, source=Web Response No relevant relationships by Jeffrey Galvin, source=Web Response No relevant relationships by Michael Luca, source=Web Response