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Rituximab for lymphoproliferative disease prior to haematopoietic stem cell transplantation for X‐linked severe combined immunodeficiency
Author(s) -
Trahair Toby N.,
Wainstein Brynn,
Manton Nicholas,
Bourne Anthony J.,
Ziegler John B.,
Rice Michael,
Russell Susan J.
Publication year - 2008
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.20887
Subject(s) - medicine , rituximab , hematopoietic stem cell transplantation , transplantation , immunosuppression , immunodeficiency , severe combined immunodeficiency , immunology , lymphoproliferative disorders , stem cell , cd20 , lymphoma , umbilical cord , haematopoiesis , primary immunodeficiency , neutropenia , oncology , immune system , chemotherapy , biochemistry , genetics , chemistry , biology , gene
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. Pediatr Blood Cancer 2008;50:366–369. © 2006 Wiley‐Liss, Inc.