Introduction:
Posttransplant lymphoproliferative disorder (PTLD) is a spectrum of disorder characterized by lymphoid or plasmacytoid proliferation secondary to extrinsic immunosuppression in solid organ or hematopoietic transplant recipients. PTLD ranges from lymphoid or plasmacytoid hyperplasia, polymorphic, monomorphic to classical lymphoma.1 While Epstein-Barr Virus (EBV) is the key driver of abnormal lymphocytic proliferation in the majority of PTLDs, EBV-negative PTLD is a distinct entity.2 The incidence of PTLD in the pediatric population is closely associated with the type of transplanted organ, with the highest incidence in lung, small bowel and heart transplant recipients. 3–6
Plasmacytoid PTLD is a rare subtype of PTLD in the pediatric population. There are few pediatric cases reported in the literature, with heterogenicities of the types of transplant, clinical manifestations, and treatment regimens.7–13 We report a case of EBV-negative, plasmacytoid PTLD in a pediatric heart transplant recipient and describe his clinical presentation, response to chemotherapy, and evolution of disease.