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

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.

Keywords::

• Lymphoma and Hodgkin disease
• chemotherapeutic approaches
• prognostication

Acknowledgements

This work was undertaken under the auspices of the CCLG and the manuscript was reviewed by the CCLG Manuscript Committee prior to submission. The CCLG is supported by a grant from Cancer Research UK (CRUK). Special thanks go to Dr. Ian Hahn, Hospital for Sick Children, Great Ormond Street, for setting up the LPD registry and to Dr. Alero Thomas, London School of Hygiene and Tropical Medicine, for performing the EBV studies. We are also grateful for NHS funding to the NIHR Biomedical Research Centre.

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