Treatment of Epstein-Barr virus lymphoproliferative disease after hematopoietic stem-cell transplantation with hydroxyurea and cytotoxic T-cell lymphocytes

Transplantation. 2004 Sep 15;78(5):755-7. doi: 10.1097/


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.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Adult
  • Brain / diagnostic imaging
  • Child, Preschool
  • Epstein-Barr Virus Infections / etiology*
  • Humans
  • Hydroxyurea
  • Lymphocyte Transfusion / adverse effects*
  • Lymphoproliferative Disorders / etiology*
  • Mucopolysaccharidosis I / therapy
  • Stem Cell Transplantation / adverse effects*
  • T-Lymphocytes / transplantation
  • T-Lymphocytes, Cytotoxic / immunology
  • Tomography, X-Ray Computed


  • Hydroxyurea