EBV-MEDIATED POSTTRANSPLANT LYMPHOPROLIFERATIVE DISORDER PRESENTING AS A PLEURAL EFFUSION AND ACUTE CELLULAR REJECTION: A CASE REPORT

Abstract

SESSION TITLE: Tuesday Fellows Case Report Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/22/2019 01:00 PM - 02:00 PM INTRODUCTION: Post-transplant lymphoproliferative disorder (PTLD) is a rare complication following adult lung transplantation, but carries a three-fold increase in the risk of death compared to non-sufferers (1). Described manifestations of PTLD include lung nodules & infiltrates, thoracic adenopathy, GI involvement and CNS mass lesions (2). CASE PRESENTATION: A 59 year-old male with a history of cystic fibrosis underwent bilateral lung transplantation for progressive hypoxic respiratory failure needing VV-ECMO; he suffered no significant complications until he developed fatigue and a 600mL drop in FEV1 two months post-transplant. A right lung opacity was found on CXR; he received antibiotics for pneumonia, but his symptoms persisted. Chest CT demonstrated mediastinal and hilar lymphadenopathy, and a large right sided effusion (Image 1). Flow cytometry on pleural fluid obtained from thoracentesis showed kappa-light-chain restricted plasmacytic cells, consistent with PTLD. An EBV PCR returned positive (584cpy/mL). He underwent bronchoscopy with transbronchial lung biopsy, endobronchial ultrasound-guided transbronchial lymph node sampling and tunneled pleural catheter placement for recurrent effusion. Pleural fluid again showed plasmacytoid cells. The transbronchial biopsy showed perivascular mononuclear cell infiltrates with endothelialitis and septal extension, concerning for grade A3 rejection. His EBV viremia worsened (2660cpy/mL). After a multidisciplinary discussion, it was decided his pathologic findings were due to PTLD; he was treated with reduction of immunosuppression and four weeks of rituximab. After the first dose, his EBV load dropped by almost half, and his FEV1 recovered. After four doses, his serum EBV was undetectable, and repeat CT showed near-resolution of the adenopathy (Image 2). DISCUSSION: Cystic fibrosis patients are at higher risk for PTLD, likely because they are younger at transplant and therefore tend to be EBV seronegative when transplanted (3). Adult EBV seropositivity is pervasive, and lung tissue is lymphoid-rich (2); transplanted lungs may carry a high load of infected B-cells to the recipient, who then receive therapies to suppress host immunity (including cytotoxic T-cells that check the infected lytic-phase B-cells which perpetuate infection) (2). While treatments differ by center, the anti-CD20 monoclonal antibody rituximab is often used to deplete B-cells, in tandem with reducing immunosuppression (2). Remission rates range from 52-66%, with better outcomes for early-onset PTLD and EBV positive patients (2). CONCLUSIONS: Our patient was unique in manifesting with a pleural effusion as a primary site of his PTLD, though he also had lymphadenopathy that regressed with therapy. While older and a cystic fibrosis patient, he had positive prognostic factors (early presentation, EBV-positivity), and ultimately had a good outcome from rituximab and reduced immunosuppresion. Reference #1: Zaffiri L, Neely M, Cherikh W, et al. Incidence and Outcomes of Post-lymphoproliferative Disorder in Lung Transplant Recipients. Analysis of the ISHLT Registry. J Heart Lung Transplant 2018; 37(4): S450. Reference #2: Green M and Michaels MG. Epstein-Barr Virus Infection and Posttransplant Lymphoproliferative Disorder. Am J Transplant 2013; 13: 41-45. Reference #3: Lowery EM, Adams W, Grim SA, et al. Increased risk of PTLD in lung transplant recipients with cystic fibrosis. J Cyst Fibrosis 2017; 16: 727-734. DISCLOSURES: No relevant relationships by Luis Angel, source=Web Response No relevant relationships by Melissa Lesko, source=Web Response No relevant relationships by Sunil Nair, source=Web Response