1/76. Rapidly developing T-cell posttransplantation lymphoproliferative disorder.Posttransplantation lymphoproliferative disorder (PTLD) of T-cell origin has been rarely described in chronically immunosuppressed allograft recipients. We report a case of renal PTLD of a T lineage that occurred shortly after transplantation under a triple-immunosuppressive regimen. Renal graft biopsy performed 58 days posttransplantation showed extensive interstitial infiltrates of polymorphic lymphoid cells, which expressed the UCHL-1 and CD3 markers for T-cell lineage. The clonal nature of the T cells in renal tissue was identified by showing rearrangement of the T-cell receptor gamma chain genes using a polymerase chain reaction (PCR). dna extracted from the graft biopsy specimen did not show the sequences of human T-cell leukemia virus type 1 (HTLV-1) by PCR. Signals for Epstein-Barr virus (EBV) infection in renal tissue were not shown by in situ hybridization. After the reduction of immunosuppressive therapy, regression of PTLD lesion and development of rejection were shown in the second graft biopsy and graftectomy specimen. The extreme rarity of rapidly developing T-cell PTLD in a renal allograft prompted us to write this report.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
2/76. Epstein-Barr virus-associated lymphoproliferative skin lesion with recurrent necrotic papulovesicles of the face.A 24-year-old man showed recurrent necrotic papulovesicles of the face. The cutaneous lesions started with erythema, followed by vesicles, necrosis, and crusting, and leaving depressed scars. light avoidance did not prevent the development of the lesions. Histologic examination revealed lymphoproliferative lesions confined to the skin. Latent Epstein-Barr virus (EBV) infection was detected in the lymphoid cells from the skin lesions by in situ hybridization. A moderate to high dosage of corticosteroids suppressed the development of new skin lesions, but relapses occurred when these systemic corticosteroids were tapered.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
3/76. Recurrent Epstein-Barr virus-associated post-transplant lymphoproliferative disorder: report of a patient with histologically similar but clonally distinct metachronous abdominal and brain lesions.A liver transplant patient developed a single central nervous system (CNS) intraparenchymal lesion 5 months after the diagnosis of an intraabdominal diffuse large B-cell post-transplant lymphoproliferative disorder (PTLD). biopsy of the new CNS lesion showed a diffuse large B-cell PTLD morphologically and immunohistochemically indistinguishable from the abdominal lesion. In addition, both lesions were positive for Epstein-Barr virus (EBV) dna by polymerase chain reaction (PCR) and for EBV-encoded rna by in situ hybridization. Although these results were consistent with a metastatic origin for the CNS lesion, the finding of an intraparenchymal lesion without leptomeningeal or dural spread was suggestive of a new primary CNS lymphoma. Proof that the brain lesion was a second primary and not a metastasis was obtained by immunoglobulin gene rearrangement studies and assessment of EBV clonality. Multiple primary lymphoid neoplasms arise at higher frequency in the setting of immunosuppression, and molecular investigations of tumor clonality can provide clinically relevant staging and prognostic information.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
4/76. 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) |
5/76. EBV infection induced transformation of benign T lymphoproliferative state in patient with chronic active EBV infection into malignant lymphoma: implication of EBV infection as additive oncogenic factor in tumorigenesis.An 11-year-old girl with chronic EBV (Epstein-Barr virus) infection, who later developed malignant lymphoma in the lung, is reported. She had an increased number of V alpha2, V beta8, CD3, CD4, and HLADR positive activated lymphocytes (20-30% of total lymphocytes) in peripheral blood. One year later, she developed lymphoma in the lung, which was V alpha2, V beta8, CD3, CD4, HLADR and IL2Rbeta positive. At that time, the population in the peripheral blood increased up to 40%, but there was no evidence of lymphoma in the bone marrow. in situ hybridization revealed lymphoma cells were EBER-1 positive but gp350/220 and LMP mRNA negative. The EBV genome was detected in the tumor, but not in the peripheral T cells. Clonal analysis of the lymphoma cells revealed monoclonal rearrangement of the TcRbeta and gamma gene, however, investigation of the terminal repeat of EBV gene did not show the monoclonal pattern. These results indicate that infection of EBV into clonally activated T cells was related with transformation from benign lymphoproliferative disease to malignant lymphoma in this patient.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
6/76. Epstein-Barr virus-associated high-grade anaplastic plasmacytoma in a renal transplant patient.Allograft transplant patients have an increased risk of developing polyclonal or monoclonal lymphoproliferative disorders, but high-grade anaplastic plasmacytomas are extremely rare in these patients. We present a renal transplant patient who developed multiple extramedullary high-grade anaplastic plasmacytomas in the oral cavity, the left maxillary antrum, the scalp, the thigh and the upper abdominal wall with no evidence of diffuse bone marrow infiltration. Epstein-Barr virus (EBV) mRNA transcripts were detected within the myeloma cells by in situ hybridization using EBER1-2 probes. Following discontinuation of immunosuppression applied, the patient was treated with a cyclophosphamide-prednisone regimen followed by local irradiation, and a complete remission was achieved within four weeks. We concluded that EBV-associated high-grade anaplastic plasmacytomas constitute one more type of post-transplant lymphoproliferative disorder, and that despite their characterization as highly malignant neoplasms, their clinical behavior is not always aggressive.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
7/76. Large deletion of the X-linked lymphoproliferative disease gene detected by fluorescence in situ hybridization.The X-linked lymphoproliferative disease (XLP) is an inherited immunodeficiency characterized by an abnormal responses to infection with Epstein-Barr virus (EBV), resulting in fatal infectious mononucleosis, hypogammaglobulinemia, virus-associated hemophagocytic syndrome, and malignant lymphoma. Mutations in the gene coding for a T cell-specific SLAM-associated protein (SAP) have been recently identified in XLP patients. We report on a 1-year-old boy representing fulminant hemophagocytic syndrome. He developed high fever, lymphadenopathy, hepatosplenomegaly with liver dysfunction, and pancytopenia with marrow hemophagocytosis. EBV dna was abnormally increased in the blood. polymerase chain reaction failed to amplify SAP mRNA and genomic dna products from the patient' As peripheral blood. A large deletion of the SAP gene was confirmed by fluorescence in situ hybridization (FISH). FISH analysis also disclosed that the patient's mother was a carrier. We conclude that FISH can be useful in the diagnosis of XLP with large deletions of the SAP gene and its carrier state.- - - - - - - - - - ranking = 5keywords = hybridization (Clic here for more details about this article) |
8/76. Fulminant EBV( ) T-cell lymphoproliferative disorder following acute/chronic EBV infection: a distinct clinicopathologic syndrome.This study describes the clinicopathologic features of 5 patients who developed a fulminant Epstein-Barr virus (EBV)-positive clonal T-cell lymphoproliferative disorder (LPD) after acute EBV infection. One additional patient developed a similar disorder in the setting of long-standing chronic active EBV infection. Detailed immunophenotyping, in situ hybridization for EBV early rna-1 (EBER1) and polymerase chain reaction (PCR) analyses for immunoglobulin (Ig) heavy chain and T-cell receptor (TCR)-gamma gene rearrangements were performed on paraffin-embedded tissue from all patients. In addition, EBV strain typing and detection of the characteristic 30-bp deletion of the latent membrane protein-1 (LMP-1) gene were performed by PCR. Controls included 8 cases of uncomplicated infectious mononucleosis (IM). patients included 4 males and 2 females with a median age of 18 years (2-37 years). Three patients were Mexican, 2 were white, and 1 was of Asian descent. All presented with fever, hepatosplenomegaly, and pancytopenia; 5 were previously healthy, but had a clinical history of a recent viral-like upper respiratory illness (1 week to 2 months), and 1 patient had documented chronic active EBV infection for 7 years. Serologic data for EBV were incomplete but titers were either negative or only modestly elevated in 3 cases. In 1 case serology was consistent with severe chronic active EBV infection. In the remaining 2 cases serologic studies were not performed. All patients died within 7 days to 8 months of presentation with T-cell LPD. On histologic examination, the liver and spleen showed prominent sinusoidal and portal lymphoid infiltrates of CD3( ), beta F1( ), EBER1( ) T cells lacking significant cytologic atypia. Two cases were CD4( ), 2 cases were CD8( ), and 2 cases had admixed CD4( ) and CD8( ) cells without clear subset predominance. All were TIA-1( ), CD56(-). Only rare B cells were noted. Marked erythrophagocytosis was present. Molecular analysis revealed identical T-cell clones in 2 or more sites (liver, spleen, lymph node) in 5 cases. All patients carried type A EBV; 4 cases had wild-type EBV-LMP, and 2 showed the 30-bp deletion. This fulminant T-cell LPD after acute/chronic EBV infection is characterized by hepatosplenomegaly, often without significant lymphadenopathy, fever, liver failure, pancytopenia, and erythrophagocytosis indicative of a hemophagocytic syndrome. EBV serology may be misleading, with lack of elevated titers. The presence of an EBER1( ) T-cell infiltrate with scant B cells should alert one to this diagnosis. Although cytologic atypia is minimal, studies for T-cell clonality confirm the diagnosis. (blood. 2000;96:443-451)- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
9/76. Early post-transplant lymphoproliferative disease following heart transplantation in the absence of lymphocytolytic induction therapy.We report a case of post-transplant lymphoproliferative disease presenting as a disseminated polymorphous B-cell lymphoma involving the cardiac allograft 3 months following transplantation in a recipient who did not receive anti-lymphocyte induction immunosuppression. in situ hybridization for the lytic Epstein-Barr virus marker NOT I was positive within a lymphocytic infiltrate on endomyocardial biopsy. Our case is the third of early post-transplant lymphoproliferative disease (within 6 months of transplantation) involving the heart allograft in the absence of anti-lymphocyte induction immunosuppression. Post-transplant lymphoproliferative disease of the heart allograft should be considered in the presence of an atypical cardiac lymphocytic infiltrate, with possible differentiation from allograft rejection using in situ hybridization for Epstein-Barr virus.- - - - - - - - - - ranking = 2keywords = hybridization (Clic here for more details about this article) |
10/76. Post-transplant lymphoproliferative disorders (PTLD) after renal transplantation: management and evolution of seven cases among 1002 renal transplants in Sao Paulo, brazil.We reported seven cases (0.7%) of PTLD among 1002 renal transplants performed at Renal Transplant Service from Hospital Sao Paulo-Universidade Federal de Sao Paulo/Escola Paulista de Medicina, Sao Paulo, brazil, between 1976 and 1997. There were three male and four female patients with median age of 37 year-old. According to Ann Arbor staging system there were four localized extra-nodal intermediate-grade NHL, one disseminated low-grade NHL and two polyclonal lymphoid hyperplasia. Four patients received cadaveric, two received related and one received unrelated renal transplant. PTLD occurred after a median latency period of 36 months (ranging from 5 to 84 months). in situ hybridization for EBER1 was performed in five patients and molecular evidence of EBV was found in 3 cases (two DLCL and one lymphoplasmocytoid lymphoma). All patients were treated with immunosuppression withdrawal, four patients received anthracyclin-based chemotherapy for control of localized or systemic clonal disease and three were treated with resection of primary PTLD. Four of seven patients (57%) are in complete remission 11, 20, 25 and 79 months after PTLD onset. One patient lost follow-up and two patients died due to lymphoma relapse, respectively 4 and 10 months after completion of treatment. In conclusion, our experience with this small group of patients showed that: 1) immunosuppression withdrawal is not necessarily associated with loss of renal transplant and can be used as the only treatment for polyclonal PTLD; 2) chemotherapy can simultaneously lead to clonal PTLD remission and periodic immunosuppression, avoiding graft rejection after immunosuppression withdrawal.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
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