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Blood, 15 September 2007, Vol. 110, No. 6, pp. 2209-2214. Prepublished online as a Blood First Edition Paper on May 14, 2007; DOI 10.1182/blood-2006-12-062174. This Article Full Text Full Text (PDF) All Versions of this Article: blood-2006-12-062174v1 110/6/2209    most recent Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Cohen, J. M. Articles by Veys, P. Search for Related Content PubMed PubMed Citation Articles by Cohen, J. M. Articles by Veys, P. Related Collections Immunobiology Neoplasia Transplantation Clinical Trials and Observations Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  TRANSPLANTATION Successful treatment of lymphoproliferative disease complicating primary immunodeficiency/immunodysregulatory disorders with reduced-intensity allogeneic stem-cell transplantation Jonathan M. Cohen1,2, Neil J. Sebire3, Julia Harvey1, H. Bobby Gaspar1,5, Cale Cathy1, Alison Jones1, Kanchan Rao4, David Cubitt6, Persis J. Amrolia4,5, E. Graham Davies1,5, and Paul Veys4 1 Department of Clinical Immunology, Great Ormond Street Hospital, London; 2 Infectious Diseases and Microbiology Unit, Institute of Child Health, University College London, Departments of 3 Histopathology and 4 Bone Marrow Transplantation, Great Ormond Street Hospital, London; 5 Molecular Immunology Unit, Institute of Child Health, University College London, London; 6 Department of Virology, Great Ormond Street and Hospital, London, United Kingdom Lymphoproliferative disease (LPD) is a recognized complication of primary immunodeficiency (PID) and immunodysregulatory syndromes. Historically, it has a very poor outcome. For patients surviving LPD, myeloablative hematopoietic stem cell transplantation (SCT) was the only cure for the underlying PID, with a high risk of developing posttransplantation complications, including recurrent lymphoproliferative disease. We describe 8 patients with a range of PID and immunodysregulatory syndromes complicated by LPD. After initial treatment of the LPD (including the use of anti-CD20 monoclonal antibody, rituximab, in 6 of the patients), all patients underwent reduced-intensity conditioning (RIC) SCT with prospective monitoring for Epstein-Barr virus (EBV) viremia. After transplantation, 3 patients received rituximab, and 3 patients received prophylactic EBV-specific cytotoxic T-lymphocytes. Only 1 patient developed recurrent LPD posttransplantation, which responded to rituximab. All patients who underwent transplantation survive free of LPD and are cured of their PID at a median follow-up of 4 years (range, 1-7 years). With careful monitoring and pre-emptive therapy, we advocate this RIC SCT approach to patients with PID who have pre-existing EBV-LPD. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: H. E. Heslop How I treat EBV lymphoproliferation Blood, November 5, 2009; 114(19): 4002 - 4008. [Abstract] [Full Text] [PDF] M. Bosticardo, F. Marangoni, A. Aiuti, A. Villa, and M. Grazia Roncarolo Recent advances in understanding the pathophysiology of Wiskott-Aldrich syndrome Blood, June 18, 2009; 113(25): 6288 - 6295. [Abstract] [Full Text] [PDF]

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