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Type: Journal article
Title: Rituximab for lymphoproliferative disease prior to haematopoietic stem cell transplantation for X-linked severe combined immunodeficiency
Author: Trahair, T.
Wainstein, B.
Manton, N.
Bourne, A.
Ziegler, J.
Rice, M.
Citation: Pediatric Blood & Cancer, 2008; 50(2):366-369
Publisher: Wiley-Liss
Issue Date: 2008
ISSN: 1545-5009
Statement of
Toby N. Trahair, Brynn Wainstein, Nicholas Manton, Anthony J. Bourne, John B. Ziegler, Michael Rice and Susan J. Russell
Abstract: Lymphoproliferative disease (LPD) is a complication of congenital and acquired immunodeficiency states. There are a number of treatment options for LPD arising after haematopoietic stem cell or solid organ transplantation including reduction of immunosuppression, targeted therapies, such as the anti-CD20 monoclonal antibody, rituximab, and EBV specific cytotoxic lymphocytes. Treatment of LPD in children with congenital immunodeficiency syndromes remains unsatisfactory and is associated with a high mortality rate. We recently managed an infant found to have polymorphic LPD concurrent with X-linked severe combined immunodeficiency (SCID). Haematopoietic stem cell transplantation (HSCT) had to be deferred because of progressive LPD. Treatment with rituximab resulted in regression of the LPD following which the patient received a 5/6 HLA matched umbilical cord blood (UCB) transplant. The patient remains well 20 months following transplantation. Rituximab treatment may have a useful role in the control of LPD associated with congenital immunodeficiency prior to HSCT.
Keywords: cord blood transplantation; lymphoproliferative disease; rituximab; severe combined immunodeficiency
Description: Copyright © 2008 Wiley-Liss, Inc., A Wiley Company
RMID: 0020080055
DOI: 10.1002/pbc.20887
Appears in Collections:Paediatrics publications

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