Samart PakakasamaGretchen M. EamesMichael C. MorrissM. Helen HulsCliona M. RooneyHelen E. HeslopRobert A. KranceMahidol UniversityCook Children's Medical CenterTexas Children's Hospital HoustonBCM Center for Cell and Gene Therapy2018-07-242018-07-242004-09-15Transplantation. Vol.78, No.5 (2004), 755-757004113372-s2.0-4544344289https://repository.li.mahidol.ac.th/handle/123456789/21539Epstein-Barr virus (EBV) lymphoproliferative disease (LPD) is a potentially fatal complication that may follow allogeneic hematopoietic stem-cell transplantation (HSCT). In this article, the authors report a 2-year-old girl with Hurler's syndrome who developed multiple central nervous system (CNS) EBV LPD lesions 1 year after unrelated donor HSCT. Before this CNS occurrence, the patient had a complete response to rituximab treatment for EBV LPD of the spleen and lymph nodes; however, treatment of the CNS disease with rituximab proved ineffective. Because of reported favorable response of primary CNS EBV LPD in two human immunodeficiency virus-positive patients, the authors treated this patient with low-dose oral hydroxyurea. The patient improved clinically, with a decrease in size of multiple EBV LPD brain lesions. Subsequently, the patient received EBV-specific cytotoxic T-cell lymphocytes and remains well. The benefit and limited toxicity of hydroxyurea therapy merit its further consideration as treatment for EBV LPD.Mahidol UniversityMedicineArticleSCOPUS10.1097/01.TP.0000129813.54517.25