Tonsillar Post-transplant Lymphoproliferative Disorder Presenting With Hemoptysis After Heart Transplantation: A Case Report and Review of the Literature.

Post-transplant lymphoproliferative disorder (PTLD) is a potentially fatal complication of solid organ transplantation. Heart transplant recipients are among those at elevated risk for developing PTLD. Tonsillar involvement is a recognized manifestation, typically presenting as recurrent tonsillitis or progressive tonsillar hypertrophy, although hemorrhagic presentations are rare. We present the case of a 44-year-old man with nonischemic cardiomyopathy, status post-orthotopic heart transplantation in December 2024, who developed recurrent tonsillitis with progressive bilateral tonsillar hypertrophy in the setting of active Epstein-Barr virus (EBV) and cytomegalovirus (CMV) co-infection. He initially presented with a four-month history of throat pain and was subsequently found to have epiglottitis, for which he was treated with antibiotics, antivirals, and corticosteroids. Two months later, he presented to the emergency department (ED) with hemoptysis after a coughing episode, reporting expulsion of a large blood clot. Laboratory studies revealed a hemoglobin (Hgb) of 6.6 g/dL and leukopenia, necessitating transfusion of packed red blood cells (pRBCs). Flexible fiberoptic laryngoscopy (FFL) demonstrated blood pooling in the supraglottis, suspected to originate from the palatine tonsils. Over the following days, he developed a perforation of the right anterior tonsillar pillar with tonsillar necrosis and required additional transfusion for persistent anemia. Bilateral tonsillectomy with direct laryngoscopy was performed, revealing necrotic tonsillar tissue with sloughing extending to the lateral pharyngeal walls such that normal tissue planes were difficult to establish. Following tonsillectomy, hemoptysis resolved completely, Hgb levels stabilized, and no further transfusions were required. Final surgical pathology was consistent with polymorphic PTLD, supported by fluorescence in situ hybridization demonstrating loss of BCL6/3q27, MYC/8q, BCL2/18q21.3, and IGH/14q32. Flow cytometry was negative for overt lymphoma. A systematic literature search of PubMed/MEDLINE across 10 thematic areas identified 131 unique articles. Combined with a bibliography review, 30 references were selected for inclusion. A review of the literature revealed no previous reports of hemoptysis originating from tonsillar PTLD or resolving after tonsillectomy for PTLD. To our knowledge, this is the first reported case of hemoptysis as a presenting feature of tonsillar PTLD in an adult heart transplant recipient. This case emphasizes the importance of maintaining a high index of suspicion for PTLD in transplant recipients with persistent or progressive tonsillar disease accompanied by hemorrhagic or necrotic features, and reinforces the dual diagnostic and therapeutic role of tonsillectomy in the management of localized tonsillar PTLD.