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.