Abstract
Posttransplant lymphoproliferative disorder (PTLD) after lung transplantation (LT) occurs due to immunosuppressant therapy that limits antiviral host immunity and permits Epstein-Barr viral (EBV) replication and transformation of B cells.
We present the case of a 61-year-old man with history of bone marrow transplant (08/2009) for non-Hodgkin's lymphoma and bilateral LT (04/2021) for idiopathic pulmonary fibrosis - EBV status: D+/R+. The transplant was complicated by prolonged duration of ECMO and a lengthy post-LT hospital stay for renal failure, significant deconditioning, and acute on chronic respiratory failure requiring tracheostomy and fiberoptic bronchoscopic airway stent placement to limit atelectasis. He also required a muscle flap for right bronchial anastomotic dehiscence in this time course. After 4 months, he was finally discharged to home with tracheostomy in place, requiring oxygen supplementation based only on physical demands. On his return to clinic, an inspection bronchoscopy was performed to assess the airway stent, which was found to have purulent secretions concerning for infection. Bronchoalveolar lavage was positive for pan-sensitive Pseudomonas requiring intravenous antibiotic therapy. At the same time, a chest CT revealed multiple pulmonary nodules in the right lung base, the largest measuring 2.8 cm in diameter. A CT-guided biopsy confirmed EBV-positive polymorphic PTLD. Immunostaining yielded positive results for CD20, CD138, CD79a, MUM1, and EBV-encoded RNAs (EBER) (Figure 1). Notably, this patient never had a positive EBV-viral load. Given his undetectable EBV levels, a decision was made to reduce immunosuppression and follow imaging and blood counts as appropriate.
EBV-positive PTLD with negative EBV viremia is an unusual combination in an EBV-seropositive recipient with donor non-mismatch. The patient's tenuous respiratory status requiring intensive corticotherapy likely contributed significant risk for PTLD in this case.