Rituximab for lymphoproliferative disease prior to haematopoietic stem cell transplantation for X-linked severe combined immunodeficiency

Authors

  • Toby N. Trahair BSc(Med), MBBS, PhD, FRACP,

    1. The Centre for Children's Cancer and Blood Disorders, Sydney Children's Hospital, Sydney, NSW, Australia
    2. School of Women's and Children's Health, the University of New South Wales, Sydney, NSW, Australia
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  • Brynn Wainstein MBChB, FRACP,

    1. Department of Immunology, Sydney Children's Hospital, Sydney, NSW, Australia
    2. School of Women's and Children's Health, the University of New South Wales, Sydney, NSW, Australia
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  • Nicholas Manton MBBS, BMedSci, FRCPA,

    1. Department of Histopathology, Women's and Children's Hospital, Adelaide, SA, Australia
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  • Anthony J. Bourne MBBS, FRCPA,

    1. Department of Histopathology, Women's and Children's Hospital, Adelaide, SA, Australia
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  • John B. Ziegler MBBS, FRACP, MD,

    1. Department of Immunology, Sydney Children's Hospital, Sydney, NSW, Australia
    2. School of Women's and Children's Health, the University of New South Wales, Sydney, NSW, Australia
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  • Michael Rice MBBS, FRACP,

    1. Department of Clinical Haematology/Oncology, Women's and Children's Hospital, Adelaide, SA, Australia
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  • Susan J. Russell MBBS, FRACP

    Corresponding author
    1. The Centre for Children's Cancer and Blood Disorders, Sydney Children's Hospital, Sydney, NSW, Australia
    2. School of Women's and Children's Health, the University of New South Wales, Sydney, NSW, Australia
    • Centre for Children's Cancer and Blood Disorders, Sydney Children's Hospital, High Street, Randwick, NSW 2031, Australia.
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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. Pediatr Blood Cancer 2008;50:366–369. © 2006 Wiley-Liss, Inc.

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