title: Treatment of Epstein-Barr virus lymphoproliferative disease after hematopoietic stem-cell transplantation with hydroxyurea and cytotoxic T-cell lymphocytes.
abstract: Epstein-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.
authors.affiliation: Department of Pediatrics, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
authors.forename: Samart
authors.initials: S
authors.lastname: Pakakasama
authors.forename: Gretchen M
authors.initials: GM
authors.lastname: Eames
authors.forename: Michael C
authors.initials: MC
authors.lastname: Morriss
authors.forename: M Helen
authors.initials: MH
authors.lastname: Huls
authors.forename: Cliona M
authors.initials: CM
authors.lastname: Rooney
authors.forename: Helen E
authors.initials: HE
authors.lastname: Heslop
authors.forename: Robert A
authors.initials: RA
authors.lastname: Krance
mesh_terms: D000328:Adult; D001921:Brain / Q000000981:diagnostic imaging; D002675:Child, Preschool; D020031:Epstein-Barr Virus Infections / Q000209:etiology*; D006801:Humans; D006918:Hydroxyurea; D017710:Lymphocyte Transfusion / Q000009:adverse effects*; D008232:Lymphoproliferative Disorders / Q000209:etiology*; D008059:Mucopolysaccharidosis I / Q000628:therapy; D033581:Stem Cell Transplantation / Q000009:adverse effects*; D013601:T-Lymphocytes / Q000637:transplantation; D013602:T-Lymphocytes, Cytotoxic / Q000276:immunology; D014057:Tomography, X-Ray Computed
chemical_list: D006918:Hydroxyurea