title: Successful treatment of central nervous system PTLD with rituximab and cranial radiotherapy.
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.
  
  
  METHODS
  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.
authors.affiliation: Department of Paediatric Nephrology, Great Ormond Street Hospital NHS Trust, London, UK. valerie.said@gov.mt
authors.forename: Valerie
authors.initials: V
authors.lastname: Said-Conti
authors.forename: Persis J
authors.initials: PJ
authors.lastname: Amrolia
authors.forename: Mark N
authors.initials: MN
authors.lastname: Gaze
authors.forename: Sara
authors.initials: S
authors.lastname: Stoneham
authors.forename: Neil
authors.initials: N
authors.lastname: Sebire
authors.forename: Rukshana
authors.initials: R
authors.lastname: Shroff
authors.forename: Stephen D
authors.initials: SD
authors.lastname: Marks
mesh_terms: D058846:Antibodies, Monoclonal, Murine-Derived / Q000008:administration & dosage*; D018951:Antigens, CD20 / Q000378:metabolism; D000970:Antineoplastic Agents / Q000008:administration & dosage*; D001706:Biopsy; D016543:Central Nervous System Neoplasms / Q000175:diagnosis / Q000276:immunology / Q000628:therapy* / Q000821:virology; D059248:Chemoradiotherapy; D002648:Child; D016371:Cranial Irradiation; D004359:Drug Therapy, Combination; D020031:Epstein-Barr Virus Infections / Q000175:diagnosis / Q000821:virology*; D006801:Humans; D007150:Immunohistochemistry; D007166:Immunosuppressive Agents / Q000009:adverse effects; D007262:Infusions, Intravenous; D007676:Kidney Failure, Chronic / Q000601:surgery*; D016030:Kidney Transplantation / Q000009:adverse effects*; D016393:Lymphoma, B-Cell / Q000175:diagnosis / Q000276:immunology / Q000628:therapy* / Q000821:virology; D008279:Magnetic Resonance Imaging; D008297:Male; D000069283:Rituximab; D012640:Seizures / Q000821:virology; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D018951:Antigens, CD20; D000970:Antineoplastic Agents; D007166:Immunosuppressive Agents; D000069283:Rituximab