Lymphoproliferative disorders after organ transplantation in children

Background. After organ transplant, patients are at risk of posttransplant lymphoproliferative disorders (PTLD), The purpose of this study was to anal yze 26 pediatric cases of PTLD observed at our institution between 1988 and 1996, and to evaluate the validity of the Society for Hematopathology Work shop (SHPW) 1997 classification in our patient population. Methods, Charts were reviewed for analysis of incidence, clinical course, a nd outcome. Tissue samples were classified by a pathologist according to SH PW recommendations. Results, By morphology, 20 were monomorphic, 5 polymorphic, and 1 hyperplas tic, Assessment of lineage by morphology, molecular studies, and immunophen otyping did not correlate in six cases. By immunophenotyping, 12 were B cel l, 4 T cell, 8 mixed B/T cells, and 2 undetermined. The 20 patients evaluab le for treatment efficacy were treated with various therapeutic combination s, including immunosuppressive drug reduction, acyclovir/ganciclovir, inter feron-alpha, immunoglobulins, surgery, and local irradiation. No patient re ceived systemic chemotherapy, Thirteen patients achieved complete remission and 3, partial; 1 died 5 days after starting therapy, and 3 of progressive disease. Adverse prognostic factors included low platelet or neutrophil co unts; stage III-IV and SHPW morphology were marginally significant. Conclusions, The majority of patients eligible for treatment can be cured w ith immunosuppressive drug reduction and antiviral drugs, along with surger y and irradiation when indicated. Systemic chemotherapy or innovative appro aches may have a role in unresponsive cases. Morphologic SHPW grouping is f easible and seems to have clinical relevance. However, correlation with clo nality and immunophenotyping is not always possible, necessitating modifica tions including segregation of descriptive morphology from clonality and ce ll origin.