High incidence of posttransplant lymphoproliferative disease in pediatric patients with cystic fibrosis

A major cause of morbidity and mortality following lung transplantation is posttransplant lymphoproliferative disease (PTLD). In a retrospective cohor t analysis of pediatric patients, we evaluated the risk factors associated with PTLD in 128 first-time lung transplant recipients from 1990 to 1997. T he greatest risk factor for PTLD was a diagnosis of cystic fibrosis (CF). O f the 16 patients in our analysis who had PTLD, 13 had a diagnosis of CF (o dds ratio [OR]: 5.8; confidence interval 95% [CI]: 1.6 to 21.4). Because of the high frequency of PTLD in patients with CF (13 of 61; 23%), we perform ed a retrospective cohort analysis in which patients with CF and PTLD were designated as cases and patients with CF and without PTLD served as control s. In patients with CF, the only risk factor associated with PTLD was two o r more episodes of acute rejection within 3 mo after transplantation (OR: 1 1.0; 95% CI: 2.7 to 55.7). Age, recipient Epstein-Barr virus or cytomegalov irus status, induction with antilymphocyte globulin or antithymoyte globuli n (ATG), or use of ATG or OKT3 for acute rejection episodes were not risk f actors for PTLD. The high frequency of PTLD in the subgroup of patients wit h two or more episodes of graft rejection within 2 mo after lung transplant ation was unexpected, and warrants further investigation in prospective cli nical studies and basic laboratories.