Epstein-Barr VirusAssociated 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: 5.15). 11/1993; 17(10):1046-53. DOI: 10.1097/00000478-199310000-00010
Source: PubMed


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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    • "Whilst most are high-grade B-cell non-Hodgkin's lymphoma (NHLs), a few are classical Hodgkin's lymphomas. Rare cases have also been shown to be either of T-cell or NK-cell lineages [1] [2]. T-cell neoplasms constitute 10% to 15% of all PTLDs, and about 75% of T-cell PTLDs, have been shown to be negative for EBV and to behave more aggressively. "
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    ABSTRACT: Posttransplant lymphoproliferative disorders (PTLDs) are a group of diseases that range from benign polyclonal to malignant monoclonal lymphoid proliferations. They arise secondary to treatment with immunosuppressive drugs given to prevent transplant rejection. Three main pathologic subsets/stages of evolution are recognised: early, polymorphic, and monomorphic lesions. The pathogenesis of PTLDs seems to be multifactorial. Among possible infective aetiologies, the role of EBV has been studied in depth, and the virus is thought to play a central role in driving the proliferation of EBV-infected B cells that leads to subsequent development of the lymphoproliferative disorder. It is apparent, however, that EBV is not solely responsible for the "neoplastic" state. Accumulated genetic alterations of oncogenes and tumour suppressor genes (deletions, mutations, rearrangements, and amplifications) and epigenetic changes (aberrant hypermethylation) that involve tumour suppressor genes are integral to the pathogenesis. Antigenic stimulation also plays an evident role in the pathogenesis of PTLDs. Plasmacytoid dendritic cells (PDCs) that are critical to fight viral infections have been thought to play a pathogenetically relevant role in PTLDs. Furthermore, regulatory T cells (Treg cells), which are modulators of immune reactions once incited, seem to have an important role in PTLDs where antigenic stimulation is key for the pathogenesis.
    Full-text · Article · Apr 2012 · Advances in Hematology
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    • "By combining techniques, the precise involvement of EBV in PTTLs could be more accurately assessed. Treatment of PTTLs (such as decrease or discontinuation of immunosuppressive therapy, chemotherapy, and treatment with acyclovir, ganciclovir, IFN-a, and antibodies to CD21 and CD24) has been used alone or in combination with different outcomes12346789 . Our patient was treated with surgical removal of the tumor, decrease in immunosuppressive therapy (discontinuation of azathioprine treatment), and chemotherapy with a good result. "
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    ABSTRACT: Posttransplantation T cell lymphomas (PTTLs) are rather unusual, and their etiology remains unclear. We describe a case of Epstein-Barr virus (EBV)-associated small bowel T cell lymphoma in a patient 5 years after kidney transplantation. EBV was detected in a biopsy sample by in situ hybridization, immunohistochemical staining, and polymerase chain reaction analysis. Eight previously reported cases of EBV-associated PTTL are reviewed, paying special attention to the methods used for assessing EBV. We believe that our case of EBV-associated PTTL is the most completely studied from the point of view of the methods used for detection of EBV. The prognosis of PTTL is poor, but it has been reported that therapeutic approaches can be successful if they are given early in the course of the illness. Therefore, it is necessary to improve the diagnosis PTTL and to assess the precise involvement of EBV in posttransplantation lymphoproliferative disorders.
    Preview · Article · Apr 2000 · Clinical Infectious Diseases
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    ABSTRACT: Published reports of posttransplant myeloma are extremely uncommon (three cases); to the best of our knowledge there have been no reported cases in cardiac transplant recipients. We are also unaware of any report of Epstein-Barr virus (EBV) genome studies in posttransplant myeloma. We report here the case of a 48-year-old man who developed multiple myeloma 1.5 years after cardiac transplantation. The results of a serum analysis were consistent with past EBV infection. Biopsy of a skull lesion showed a monomorphous population of malignant immature plasma cells that showed monotypic cytoplasmic staining with antibodies to lambda light chains. A monoclonal immunoglobulin heavy chain gene rearrangement was detected by polymerase chain reaction (PCR). Both EBER-1 in situ hybridization and EBNA-1 PCR were negative for the EBV genome. Cyclosporin withdrawal was followed by transient clinical and biological improvement, but the tumor later progressed and eventually stabilized in response to treatment with dexamethasone alone. This case illustrates that posttransplant lymphoproliferative disorders (PTLPDs) encompass not only EBV-positive but also EBV-negative cases and not only lymphomas but also myelomas.
    No preview · Article · Jun 1994 · Human Pathlogy
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