RAPIDLY PROGRESSIVE POST-TRANSPLANT LYMPHOPROLIFERATIVE DISORDER AFTER LUNG TRANSPLANTATION

Abstract

TYPE: Case Report TOPIC: Transplantation INTRODUCTION: Posttransplant lymphoproliferative disorder (PTLD) after lung transplantation occurs due to immunosuppressant therapy, which limits antiviral host immunity and permits Epstein-Barr viral (EBV) replication and B cell transformation. CASE PRESENTATION: A 67-year-old female, bilateral lung transplant recipient (07/2019) with a history of idiopathic pulmonary fibrosis developed rapidly progressive PTLD in the early postoperative course. DISCUSSION: At 6-months after transplant, chest radiographs revealed left hilar fullness; subsequent chest CT showed evidence of fluid in the fissure, with no obvious lymphadenopathy. Chest CT performed just two weeks later revealed a left hilar mass measuring 6.1 × 2.7 cm with encasement of the left main pulmonary artery and partial collapse of the left main bronchus. Endobronchial ultrasound-guided biopsies led to the diagnosis of CD20-positive, EBV-positive PTLD. Initial PET scan demonstrated multiple hilar lymph nodes, the largest measuring 2.2 × 2.8 cm. Immunosuppression was reduced, and because the PTLD involved only one area in an EBV-mismatched bilateral lung transplant recipient, she qualified for single-agent rituximab once/week. Unfortunately, repeat imaging after five doses of rituximab demonstrated significant disease progression. She required repeat bronchoscopy with tumor debulking for a necrotic, endobronchial tumor occluding up to one-half of the left lower lobe bronchus. She started R-mini-CHOP, and after three cycles of chemotherapy, complete response was observed on the most recent PET/CT scan. CONCLUSIONS: PTLD may progress quickly, as the initial chest CT scan showing fluid collection advanced rapidly into a hilar mass. Reduction in immunosuppression, anti-CD20 biologic therapy, and chemotherapy for refractory PTLD remains an appropriate treatment paradigm. DISCLOSURE: Nothing to declare. KEYWORD: Post-lung transplant lymphoproliferative disorder