T cell lymphoma involving the graft of a multivisceral organ recipient

Posttransplant lymphoproliferative disorders are typically of B cell origin , whereas T cell lymphomas have been rarely documented. We present a case o f a non-Hodgkin's T cell lymphoma involving the intestinal graft of a multi visceral transplant patient. The patient was a 7-year-old girl who underwen t at age 5 a multivisceral transplant secondary to short gut syndrome. Base line immunosuppressive therapy consisted of FK506, methylprednisone, and my cophenolate mofetil, At 2 years posttransplant she presented with fever, di arrhea, nausea, and vomiting. Multiple endoscopic biopsies revealed a sever e intensity, diffuse and focally nodular lymphocytic infiltrate composed pr edominantly of small, monomorphic lymphoid cells with scattered plasma cell s and abundant eosinophils, Immunohistochemically, the majority of the lymp hoid cells expressed the pan T cell marker CD3, Southern blot analysis reve aled rearrangement of the T cell receptor beta chain gene, with germline co nfiguration of the heavy immunoglobulin chain gene, confirming a clonal T c ell genotype. In situ hybridization for Epstein Barr virus revealed rare po sitive lymphoid cells, that were negative with CD3 by immunohistochemical s taining. A detailed clinico-radiological work-up revealed no other sites of involvement by the lymphomatous process. After the diagnosis of posttransp lant lymphoproliferative disorder, immunosuppression was reduced with a sub sequent partial improvement in the endoscopic appearance of the graft and a focal decrease in the lymphocytic infiltrate seen in the follow-up biopsie s. Repeat gene rearrangement studies demonstrated germline configuration of both the T cell receptor beta chain gene and the heavy chain immunoglobuli n, gene. To our knowledge, this represents the first description of a T cel l lymphoma affecting the intestinal allograft of a multivisceral transplant patient.