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.
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.
Royal Marsden Hospital, Sutton, Surrey, UK. mary.taj@icr.ac.uk
Mary M
MM
Taj
Nedim
N
Hadzic
Susan E
SE
Height
Andrew
A
Wotherspoon
Margaret
M
Burke
Rachel
R
Hobson
Maria
M
Viskaduraki
C Ross
CR
Pinkerton
D000293:Adolescent; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D005500:Follow-Up Studies; D004854:Herpesvirus 4, Human; D006801:Humans; D007165:Immunosuppression Therapy / Q000009:adverse effects* / Q000379:methods; D007223:Infant; D008206:Lymphatic Diseases; D008232:Lymphoproliferative Disorders / Q000175:diagnosis / Q000209:etiology / Q000276:immunology*; D016377:Organ Transplantation / Q000009:adverse effects; D012042:Registries; D000069283:Rituximab; D016896:Treatment Outcome; D006113:United Kingdom
D058846:Antibodies, Monoclonal, Murine-Derived; D000069283:Rituximab