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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 (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 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. Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted December 11, 2006 Accepted April 24, 2007 Successful treatment of lymphoproliferative disease complicating primary immunodeficiency / immunodysregulatory disorders with reduced-intensity allogeneic stem cell transplantation Jonathan M Cohen, Neil J Sebire, Julia Harvey, H Bobby Gaspar, Cale Cathy, Alison Jones, Kanchan Rao, David Cubitt, Persis J. Amrolia, E Graham Davies*, and Paul Veys Infectious Diseases & Microbiology Unit, Institute of Child Health, University College London, London, United Kingdom Department of Histopathology, Great Ormond Street Hospital NHS Trust, London, United Kingdom Department of Clinical Immunology, Great Ormond Street Hospital NHS Trust, London, United Kingdom Molecular Immunology Unit, Institute of Child Health, University College London, London, United Kingdom Department of Bone Marrow Transplantation, Great Ormond Street Hospital NHS Trust, London, United Kingdom Department of Virology, Great Ormond Street Hospital NHS Trust, London, United Kingdom * Corresponding author; email: davieg1{at}gosh.nhs.uk. 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 haematopoietic stem cell transplantation (SCT) was the only cure for the underlying PID, with a high risk of developing post-transplant complications including recurrent lymphoproliferative disease. We describe eight patients with a range of PID and immunodysregulatory syndromes complicated by LPD. Following initial treatment of the LPD (including the use of anti-CD20 monoclonal antibody rituximab in six of the patients), all patients underwent reduced intensity conditioned (RIC) SCT with prospective monitoring for EBV-viraemia. After transplant, three received rituximab, and three patients received prophylactic EBV-specific cytotoxic T-lymphocytes. Only one patient developed recurrent LPD post-transplant, which responded to rituximab. All transplanted patients survive free of LPD and cured of their PID, at median follow-up of four years (range one to seven years). With careful monitoring and pre-emptive therapy, we advocate this RIC SCT approach to PID patients with 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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