1/8. 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 of a non-Hodgkin's T cell lymphoma involving the intestinal graft of a multivisceral transplant patient. The patient was a 7-year-old girl who underwent at age 5 a multivisceral transplant secondary to short gut syndrome. Baseline immunosuppressive therapy consisted of FK506, methylprednisone, and mycophenolate mofetil. At 2 years posttransplant she presented with fever, diarrhea, nausea, and vomiting. Multiple endoscopic biopsies revealed a severe intensity, diffuse and focally nodular lymphocytic infiltrate composed predominantly of small, monomorphic lymphoid cells with scattered plasma cells and abundant eosinophils. Immunohistochemically, the majority of the lymphoid cells expressed the pan T cell marker CD3. Southern blot analysis revealed rearrangement of the T cell receptor beta chain gene, with germline configuration of the heavy immunoglobulin chain gene, confirming a clonal T cell genotype. in situ hybridization for Epstein Barr virus revealed rare positive lymphoid cells, that were negative with CD3 by immunohistochemical staining. A detailed clinico-radiological work-up revealed no other sites of involvement by the lymphomatous process. After the diagnosis of posttransplant lymphoproliferative disorder, immunosuppression was reduced with a subsequent partial improvement in the endoscopic appearance of the graft and a focal decrease in the lymphocytic infiltrate seen in the follow-up biopsies. Repeat gene rearrangement studies demonstrated germline configuration of both the T cell receptor beta chain gene and the heavy chain immunoglobulin. gene. To our knowledge, this represents the first description of a T cell lymphoma affecting the intestinal allograft of a multivisceral transplant patient.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
2/8. Primary angiocentric T-cell intestinal lymphoma with Epstein-Barr virus in a 5-year-old boy.A case of peripheral T-cell lymphoma affecting the small bowel of a 5-year-old boy is reported. The cells did not form a tumoral mass but infiltrated diffusely, arranged in an angiocentric pattern and associated with numerous ulcers, one of which perforated. immunohistochemistry proved them to be CD45RO-, CD3-, and CD8-positive. CD20, CD4, and CD56 markers were negative. The presence of EBV in the lymphomatous cells was demonstrated by in-situ hybridization. polymerase chain reaction study revealed T-cell receptor (TCR) gene rearrangement. Notably the hemophagocytic syndrome present on admission reverted after surgery. The authors are not aware of a previous report of intestinal T-cell lymphoma in a child.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
3/8. Chromosomal gains at 9q characterize enteropathy-type T-cell lymphoma.Genetic alterations in enteropathy-type T-cell lymphoma (ETL) are unknown so far. In this series, 38 cases of ETL were analyzed by comparative genomic hybridization (CGH). CGH revealed chromosomal imbalances in 87% of cases analyzed, with recurrent gains of genetic material involving chromosomes 9q (in 58% of cases), 7q (24%), 5q (18%), and 1q (16%). Recurrent losses of genetic material occurred on chromosomes 8p and 13q (24% each), and 9p (18%). In this first systematic genetic study on ETL, chromosomal gains on 9q (minimal overlapping region 9q33-q34) were found to be highly characteristic of ETL. fluorescence in situ hybridization analysis on four cases of ETL, using a probe for 9q34, indicated frequent and multiple gains of chromosomal material at 9q34 (up to nine signals per case). Among 16 patients with ETL who survived initial disease presentation, patients with more than three chromosomal gains or losses (n = 11) followed a worse clinical course than those with three or less imbalances (n = 5). The observation of similar genetic alterations in ETL and in primary gastric (n = 4) and colonic (n = 1) T-cell lymphoma, not otherwise specified, is suggestive of a genetic relationship of gastrointestinal T-cell lymphomas at either localization.- - - - - - - - - - ranking = 2keywords = hybridization (Clic here for more details about this article) |
4/8. Primary intestinal posttransplant T-cell lymphoma.There have been only five reported cases of primary posttransplant T-cell lymphoma. We report the first case associated with the use of sirolimus (Rapamycin, Wyeth-Ayerst, philadelphia, PA). The patient, receiving prednisone, cyclosporine, and sirolimus treatment, developed ascites, diarrhea, and weight loss 7 months after his second renal transplant. Tissue obtained at laparotomy established the diagnosis of primary T-cell lymphoma. Latent membrane protein-1 for Epstein-Barr virus was negative, but in-site hybridization test for Epstein-Barr-encoded rna was positive. Despite aggressive chemotherapy, the patient died 8 months posttransplant. This is the sixth reported case of primary intestinal posttransplant T-cell lymphoma, but it is the first case associated with the use of sirolimus. The incidence of posttransplant lymphoproliferative disease in patients receiving sirolimus should be studied.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
5/8. enteropathy-associated t-cell lymphoma in a renal transplant patient with evidence of Epstein-Barr virus involvement.The clinical and histological findings in a 54-year-old patient with enteropathy-associated t-cell lymphoma (EATL) occurring 18 years after renal transplantation are presented. Ten years after adult-onset coeliac disease the patient developed medium to large T-cell non-Hodgkin's lymphoma of the small intestine. Epstein-Barr virus (EBV) genome was detected by polymerase chain reaction in the lymphoma tissue and localized via Epstein-Barr virus RNAs in situ hybridization to some of the tumour cells. This is the first case report of EBV-positive EATL occurring in the setting of immunosuppression.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
6/8. carcinoma of donor origin after liver-intestine transplantation in a child.Tumor-related complications after intestinal transplantation in children have been principally EBV driven post-transplant disorders. We describe the clinical course of a child, with a diagnosis of microvillus inclusion disease who received a liver and intestine allograft at the age of 9 months. His postoperative course was significant for multiple episodes of acute intestinal allograft rejection and eventually the development of post-transplant lymphoproliferative disorder (PTLD), which resolved. At 8 yr post-transplant he presented with masses in the intestine allograft mesentery and in the right lobe of the allograft liver, biopsy of which revealed a relatively undifferentiated tumor, suggestive of a carcinoma. in situ hybridization for X and Y chromosomes, revealed his tumor to be of donor origin. Treatment included debulking of the mesenteric mass with segmental enterectomy of the intestinal allograft, and stopping his immunosuppression for a period of 4 months; this resulted in complete resolution of his malignancy. immunosuppression with tacrolimus and steroids was restarted because of intestinal allograft rejection; he died suddenly of unknown causes at 17 months post-diagnosis of carcinoma. The severely immunosuppressed state produced in this patient allowed for the development of an unusual donor derived carcinoma, which resolved spontaneously with withdrawal of immunosuppression. The mechanism of such regression of tumor may be related to restitution of immunologic competence, but is yet to be determined.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
7/8. Expression of cytotoxic molecules in intestinal T-cell lymphomas. The German Study Group on Intestinal Non-Hodgkin Lymphoma.Intestinal T-cell lymphoma (ITCL) represents a subgroup of peripheral T-cell lymphomas which is thought to arise from alpha beta intraepithelial t-lymphocytes. Since these lymphocytes may contain cytotoxic molecules, the question of whether this also holds true for ITCL arises. Twenty ITCL cases were examined for the presence of granzyme B, perforin, and T-cell-restricted intracellular antigen (TIA-1)/granule membrane protein of 17 kD (GMP-17). Two molecules with restricted expression in cytotoxic cells, granzyme B and perforin, were detected by immunocytochemistry and by in situ hybridization with an isotopically labelled rna probe, respectively. Immunocytochemistry was also performed with the antibody 2G9, which recognizes two molecules, one expressed by cytotoxic cells (TIA-1) and the other found in granulocytes and cytotoxic cells (GMP-17). Granzyme B, TIA-1/GMP-17, and perforin were found in the neoplastic cells of 16/19 cases, 19/20 cases, and 16/17 cases, respectively, of ITCL, but not in the tumour cells of the control group, which consisted of intestinal B-cell lymphomas (five cases) and CD8-negative peripheral nodal T-cell lymphomas (six cases). At least one of these molecules was expressed in the tumour cells of all ITCL cases. 2G9 proved to be the most sensitive immunohistological marker, since reactivity with this antibody was not only observed in the highest number of cases, but also found in high numbers of neoplastic cells in positive cases. In conclusion, ITCL appears to show cytotoxic differentiation in all cases. In conjunction with immunophenotypic and genotypic data, our results support a uniform derivation of this tumour from intraepithelial alpha beta cytotoxic t-lymphocytes.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
8/8. Primary intestinal gamma-delta T-cell lymphoma with evidence of Epstein-Barr virus.AIMS: Primary intestinal T-cell lymphomas account for about 5% of all primary gastrointestinal lymphomas and are mostly associated with coeliac disease. They usually express the CD3-associated T-cell receptor alpha/beta heterodimer and HML1, and some are related with Epstein-Barr virus (EBV). As far as we know, the present report describes the first case of primary gamma-delta (gamma delta) EBV-associated intestinal T-cell lymphoma without enteropathy. Only hepatosplenic, nasal and cutaneous gamma delta T-cell lymphomas have previously been described. methods AND RESULTS: Our case concerned a 43-year-old man with no history of coeliac disease, who presented with multifocal small bowel involvement showing high grade T-cell lymphoma with medium sized and large pleomorphic cells and a small pleomorphic T-cell component. Angioinvasion and angiocentricity were occasionally present. Immunohistochemical studies of lymphoma cells showed a T-cell gamma delta phenotype (CD3 , CD2 , TCR delta 1 , V delta 2 and beta F1-) without expression of CD4, CD8, CD5, or HML1. Most tumour cells were positive for the cytotoxic granular proteins TiA1 and granzyme B. Rearrangement of the TCR gamma chain gene was demonstrated by polymerase chain reaction and in-situ hybridization with EBER probes revealed strong nuclear positivity in virtually all neoplastic cells. CONCLUSION: We described the first case of primary intestinal gamma delta T-cell lymphoma without enteropathy in which EBV might fulfil a pathogenic role.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |