Epstein-Barr virus-associated T-cell lymphoma in a renal transplant patient.

Department of Pathology, University of Texas Medical Branch, Galveston 77555.
American Journal of Surgical Pathology (Impact Factor: 4.59). 11/1993; 17(10):1046-53. DOI: 10.1097/00000478-199310000-00010
Source: PubMed

ABSTRACT Posttransplant lymphoproliferative disorders in organ allograft recipients are most commonly of B cell origin, whereas T cell lymphomas are rarely described. We report a case of T cell immunoblastic large cell lymphoma associated with Epstein-Barr virus (EBV) that occurred in a recipient of a cadaveric renal transplant 7 years posttransplantation. On paraffin immunophenotyping, none of the neoplastic cells stained with the T cell-associated markers used, but did show strong CD30 expression. Flow cytometric studies revealed a predominance of T cells without definite evidence of T cell neoplasia. Frozen section immunophenotyping studies revealed a T cell phenotype with aberrant expression, and genotypic studies demonstrated T cell receptor beta gene rearrangement with germline configuration of immunoglobulin heavy chain and kappa light chain genes, confirming a T lineage. EBV-encoded RNA transcripts were demonstrated within the neoplastic cells by in situ hybridization. Southern blot analysis using probes derived from the terminal repeat region of the virus detected a single restriction band indicating a clonal population. We believe this is the first case of a posttransplant T cell lymphoma in which the EBV genome has been demonstrated. This case also illustrates the pitfalls of paraffin immunophenotyping in the diagnosis of T cell lymphoma.

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    ABSTRACT: Background. Although 14% of the malignant lymphomas after organ transplantation are reported to be T-cell lymphomas, only a few cases are described in the literature.Methods. The authors presented three new cases. They summarized the clinical data and analyzed histologic and immunochemical findings. The presence of Epstein-Barr virus (EBV) and human T-cell lymphoma type 1 (HTLV-1) were investigated. T-cell receptor (TCR) rearrangement was analyzed by Southern blot technique in two cases.Results. Two of the three lymphomas developed after renal transplantation. One was a T-cell lymphoma of pleomorphic medium-sized cell type and the other was a T-cell lymphoblastic lymphoma; the third T-cell lymphoma was an anaplastic large cell (Ki-1 positive) type that developed after heart transplantation. No association was established with EBV or HTLV-1. A monoclonal TCR rearrangement was found in the two cases that were analyzed. A literature search revealed 22 other cases. Nineteen of the 22 reported cases were peripheral T-cell lymphomas. Almost all lymphomas presented in extranodal sites. The time between diagnosis and organ transplantation seemed to be influenced by the type of immunosuppressive therapy. In five cases, EBV was detected in the tumor cells. A monoclonal T-cell receptor rearrangement was found in eight cases and a polyclonal proliferation in one case. Response to therapy was variable, but often poor.Conclusions. The etiology of posttransplant T-cell lymphomas remains unclear. Similarities with post-transplant B-cell proliferations are the predominant extranodal presentation and the finding that the time of occurrence is influenced by the type of immunosuppression. In contrast with posttransplant B-cell proliferations, only a minority of the cases are associated with EBV. Most tumors appear to be monoclonal. Prognosis is generally poor, but tumor presentation with localized disease might have a somewhat better prognosis. Cancer 1994; 73:3064–72.
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