title: Rituximab for lymphoproliferative disease prior to haematopoietic stem cell transplantation for X-linked severe combined immunodeficiency.
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.
authors.affiliation: The Centre for Children's Cancer and Blood Disorders, Sydney Children's Hospital, Sydney, NSW, Australia.
authors.forename: Toby N
authors.initials: TN
authors.lastname: Trahair
authors.forename: Brynn
authors.initials: B
authors.lastname: Wainstein
authors.forename: Nicholas
authors.initials: N
authors.lastname: Manton
authors.forename: Anthony J
authors.initials: AJ
authors.lastname: Bourne
authors.forename: John B
authors.initials: JB
authors.lastname: Ziegler
authors.forename: Michael
authors.initials: M
authors.lastname: Rice
authors.forename: Susan J
authors.initials: SJ
authors.lastname: Russell
mesh_terms: D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D058846:Antibodies, Monoclonal, Murine-Derived; D018380:Hematopoietic Stem Cell Transplantation / Q000379:methods*; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use*; D007223:Infant; D008232:Lymphoproliferative Disorders / Q000150:complications / Q000188:drug therapy* / Q000276:immunology; D008297:Male; D000069283:Rituximab; D053632:X-Linked Combined Immunodeficiency Diseases / Q000150:complications / Q000276:immunology / Q000628:therapy*
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D007155:Immunologic Factors; D000069283:Rituximab