Long-term outcome for immune suppression and immune related lymphoproliferative disorder: prospective data from the United Kingdom Children's Leukaemia and Cancer Group registry 1994-2004

Mary M. Taj*, Nedim Hadzic, Sue Height, Andrew Wotherspoon, Margaret Burke, Rachel Hobson, Maria Viskaduraki, C. Ross Pinkerton

*Corresponding author for this work

    Research output: Contribution to journalArticlepeer-review

    7 Citations (Scopus)

    Abstract

    Prospective national registry data on 98 patients were studied to determine the long-term outcome of immune related lymphoproliferative disease (LPD) and define prognostic factors. Seventy-three developed LPD following organ transplant (26 liver, 21 heart, 15 kidney, nine bone marrow [BM], two bowel). Twenty-five had non-transplant related immunosuppression. Age was 1.1-17 years (median 8.6). Fifty-eight patients had lymphomatous, 21 systemic and 17 lymphadenopathic disease. Sixty (73%) were disseminated and 22 (27%) localized. Thirty-three (54%) were monoclonal. Seventy-three (83%) were Epstein-Barr virus (EBV) positive. Median follow-up was 7.6 years. LPD developed earlier after liver and BM as compared to heart or kidney transplant. Five-year overall survival (OS) was 58%. Prognosis was best after liver and kidney transplant (OS >77%). Mortality was higher following heart (2.5 times) and BM transplant (5 times). Adverse prognostic factors were disseminated or lymphomatous disease and lack of reduction of immunosuppression. With appropriate reduction of immunosuppression, rituximab and low-dose chemotherapy, long-term survival is high.

    Original languageEnglish
    Pages (from-to)842-848
    Number of pages7
    JournalLeukemia and Lymphoma
    Volume53
    Issue number5
    DOIs
    Publication statusPublished - May 2012

    Keywords

    • Lymphoma and Hodgkin disease
    • chemotherapeutic approaches
    • prognostication
    • EPSTEIN-BARR-VIRUS
    • STEM-CELL TRANSPLANTATION
    • SOLID-ORGAN TRANSPLANTATION
    • EBV REACTIVATION
    • LIVER-TRANSPLANTATION
    • RISK-FACTORS
    • VIRAL-LOAD
    • RITUXIMAB
    • DISEASE
    • CHEMOTHERAPY

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