Post-transplant lymphoproliferative disease in children

Epstein-Barr virus (EBV)-driven post-transplant lymphoproliferative disease (PTLD) is an important cause of morbidity and mortality following transpla ntation, and it occurs more frequently in children than in adults. Of 22 (5 %) children at our institution who developed tissue-proven PTLD 1-60 months (mean 16.5 months) following organ transplant, 11 died: nine of these 22 p atients developed PTLD between 1989 and 1993, and seven (78%) died: the rem aining 13 developed PTLD between 1994 and 1998, and four (31%) died (p = 0. 08). All nine patients who developed PTLD <6 months after transplant died, but II of 13 patients who manifested disease <greater than or equal to> 6 m onths after transplant survived (p = 0.0002), Ten of 11 (91%) survivors. bu t only two of eight (25%) children who died, had serologic evidence of EBV infection at the time of PTLD diagnosis (p=0.04), EBV seroconversion identi fied patients at risk for developing PTLD, but also characterized patients with sufficient immune function to survive EBV-related lymphoid proliferati on. In situ hybridization for EBER1 mRNA was diagnostically helpful because it detected EBV in tissue sections of all 20 patients with B-cell PTLD, in cluding: those with negative serology.