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Successful treatment of central nervous system PTLD with rituximab and cranial radiotherapy

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Abstract

Background

Primary central nervous system (PCNS) post-transplant lymphoproliferative disorder (PTLD) is a rare complication of solid organ transplantation and is typically an Epstein–Barr virus (EBV)-induced B-cell CD20+ lymphoma. The modalities of treatment include reduction in immunosuppression, cranial radiotherapy (CRT), intravenous and intrathecal rituximab when CD20 is expressed on B-lymphocytes and PTLD cells, and chemotherapy.

Case-Diagnosis/Treatment

We report the successful treatment of EBV-driven PCNS PTLD by reduction in immunosuppression (RI), CRT, and intravenous rituximab. Our patient was an 11-year-old boy with a living-related renal transplant for end-stage renal failure (ESRF) secondary to posterior urethral valves (PUV) and bilateral renal dysplasia (BRD) and on triple immunosuppression with prednisolone, tacrolimus, and azathioprine who had a rising EBV load, which was managed with reduction in tacrolimus dose, withdrawal of azathioprine, and introduction of mycophenolate mofetil (MMF).

Conclusions

The patient presented 7 years post-transplant with a seizure and abnormal neurology secondary to polymorphous hyperplastic lesions in the brain, which responded to rituximab and CRT.

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Authors and Affiliations

  1. Department of Paediatric Nephrology, Great Ormond Street Hospital NHS Trust, London, UK

    Valerie Said-Conti, Rukshana Shroff & Stephen D. Marks

  2. Department of Molecular Immunology, Institute of Child Health, London, UK

    Persis J. Amrolia

  3. Department of Clinical Oncology, University College Hospital/Great Ormond Street Hospital NHS Trust, London, UK

    Mark N. Gaze

  4. Department of Paediatric Oncology, University College Hospital, London, UK

    Sara Stoneham

  5. Department of Paediatric Pathology, Great Ormond Street Hospital NHS Trust, London, UK

    Neil Sebire

Authors
  1. Valerie Said-Conti
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  2. Persis J. Amrolia
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  3. Mark N. Gaze
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  4. Sara Stoneham
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  5. Neil Sebire
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  6. Rukshana Shroff
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  7. Stephen D. Marks
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Correspondence to Valerie Said-Conti.

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Said-Conti, V., Amrolia, P.J., Gaze, M.N. et al. Successful treatment of central nervous system PTLD with rituximab and cranial radiotherapy. Pediatr Nephrol 28, 2053–2056 (2013). https://doi.org/10.1007/s00467-013-2499-3

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  • DOI: https://doi.org/10.1007/s00467-013-2499-3

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