EBV lymphoproliferative disease of host origin after haploidentical stem cell transplantation

Post-transplant lymphoproliferative disease (PTLPD), due to the reactivation of Epstein-Barr virus (EBV), is a serious complication. The risk of the disorder increases with T-cell depletion methods, mismatched hematopoietic stem cell transplantation (HSCT), graft-versus-host disease (GVHD), and immunosuppression. In contrast to solid organ transplantation, where EBV is typically of recipient origin, the source of the EBV in HSCT recipients is donor-derived B-lymphocytes. In this report, we describe a 15-year-old girl who underwent HSCT from her father as treatment for acute myeloid leukemia (AML). She subsequently developed disseminated PTLPD involving multiple organ and nodal sites. Her neoplastic lymphoblasts were host-derived and refractory to rituximab treatment due to lack of CD20 expression.