Late Epstein-Barr virus infection of a hepatosplenic γδ T-cell lymphoma arising in a kidney transplant recipient

Background and Objectives. γδ T-cell lymphomas are only exceptionally observed in transplanted patients. Aim of this study was the detailed characterization of one such case. Design and Methods. The patient developed spontaneous splenic rupture six years after kidney transplantation. The splenic red pulp was infiltrated by medium-sied and large lymphoid cells with two of more nucleoli. At autopsy, similar lymphoid cells infiltrated the hepatic sinusoids. Histologic, immunologic and molecular studies were carried out. Results. By immunohistochemistry, the atypical lymphoid cells were found to express CD3, CD45 and CD43, indicating their T-lineage origin. Approximately 99% of spleen mononuclear cells (MNC) were CD3⁺, γδ TcR+, CD4-, CD8-, γβ TcR-. A clonal γδ TcR rearrangement (Vγ1-Jγ1.3/2/3-Cγ2; Vδ1-Dδ2-Jδ1) was detected. The final diagnosis was peripheral T-cell lymphoma, hepato-splenic γδ-type. EBV infection of spleen MNC was documented by molecular studies. However, in situ hybridization for EBER-1 (EBV-RNA) showed that only a minority of malignant lymphoid cells (5-7%) were EBV-infected. Interpretation and conclusions. It is concluded that EBV infection was as a late event involving an already transformed γδ T-cell clone. (C) 2000, Ferrata Storti Foundation.