1/92. 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 = liver (Clic here for more details about this article) |
2/92. Epstein-Barr virus infection resembling autoimmune hepatitis with lactate dehydrogenase and alkaline phosphatase anomaly.A 73-year-old man had fever, lymphadenopathy, granulocytopenia, thrombocytopenia, ascites, pleural effusion, liver injury, and an allergic-like skin rash. autoantibodies, such as anti-nuclear antibody, were shown, and there were lactate dehydrogenase and alkaline phosphatase anomalies and platelet-associated IgG. His liver injury resembled that in autoimmune hepatitis. He was diagnosed with Epstein-Barr virus (EBV) infection associated with autoimmunization because of his clinical course, fluctuation of anti EBV antibodies and positive EBV genome in circulating lymphocytes and serum. This case suggests a close relationship between EBV infection and autoimmunization or autoimmune-like hepatitis.- - - - - - - - - - ranking = 2keywords = liver (Clic here for more details about this article) |
3/92. Vanishing bile duct syndrome associated with chronic EBV infection.We reported here an adult patient with vanishing bile duct syndrome due to chronic EBV infection. A 22-year-old male was admitted to a nearby hospital complaining of a sore throat and jaundice. He received a high dose of prednisolone for bile stasis of acute viral hepatitis. However, the hepatitis did not improve, and he was transferred to our hospital. He had exhibited jaundice for one year as well as hemophagocytic syndrome and intestinal perforation. Subtotal intestinal resection was successfully performed. Three follow-up biopsied liver specimens indicated vanishing bile duct syndrome. Positive results of EBV-dna in his serum and mRNA of EBV by in situ hybridization of his liver indicated that massive doses of prednisolone caused chronic EBV infection and vanishing bile duct syndrome.- - - - - - - - - - ranking = 2keywords = liver (Clic here for more details about this article) |
4/92. Late Epstein-Barr virus infection of a hepatosplenic gamma delta T-cell lymphoma arising in a kidney transplant recipient.BACKGROUND AND OBJECTIVE: gd 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-sized and large lymphoid cells with two or 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( ), gammadelta TcR , CD4-, CD8-, alphabeta TcR-. A clonal gammadelta TcR rearrangement (Vgamma1-Jgamma1.3/2.3-Cgamma2; Vdelta1-Ddelta2-Jdelta1) was detected. The final diagnosis was peripheral T-cell lymphoma, hepato-splenic gammadelta-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 gd T-cell clone.- - - - - - - - - - ranking = 0.29217588011858keywords = hepatic (Clic here for more details about this article) |
5/92. Indications and results of chemotherapy in children with posttransplant lymphoproliferative disease after liver transplantation.Among 39 posttransplant lymphoproliferative diseases (PTLD) in a cohort of 450 pediatric liver transplant recipients, 3 had a malignant lymphoma, unresponsive to arrest of immunosuppression and to gancyclovir, interferon, and anti-interleukin 6 antibodies. lymphoma appeared 20, 46, and 96 months posttransplantation and 16, 43, and 90 months after primary Epstein-Barr virus infection. In one case, the patient had histological progression from plasmacytic hyperplasia PTLD, concomitant with symptomatic primary infection, to Burkitt-like lymphoma 43 months later. These three patients received five courses of chemotherapy, after a cyclophosphamide, doxorubicin, vincristine, and prednisone regimen for Burkitt-like or LH 89 scheme for Hodgkin-like PTLDs. Chemotherapy was well tolerated, and all three were free of disease and without immunosuppression 19, 14, and 4 months after chemotherapy. In Burkitt-like or Hodgkin-like PTLDs, immunomodulatory or antiviral drugs were inefficient. Chemotherapy is indicated and can be safely and successfully used. Long-term arrest of immunosuppression seems feasible without graft rejection.- - - - - - - - - - ranking = 5keywords = liver (Clic here for more details about this article) |
6/92. central nervous system involvement of Epstein-Barr virus lymphoproliferative disease in a patient with acute lymphocytic leukemia.Epstein-Barr virus-related lymphoproliferative disease (EBV-LPD) is a serious and often fatal complication of a variety of immune-suppressed conditions. A 6-year-old boy undergoing chemotherapy for standard-risk acute lymphocytic leukemia experienced separate episodes of EBV-LPD in different organ systems. The patient experienced three separate episodes of EBV-LPD in the cervical lymph node, the central nervous system (CNS), and the liver occurring, respectively, in January 1992, February 1992, and November 1993 after the completion of chemotherapy in May 1993. The EBV presence was confirmed by in situ hybridization in the biopsy samples from each lesion. Several different treatment modalities, including acyclovir, intravenous gamma globulin, and surgery were used to combat the EBV-LPD. The patient has recovered completely, with normal CNS and liver function, and for the past 6 years has experienced leukemia remission while not receiving chemotherapy. Careful monitoring of patients and the use of new immune therapies offer the highest chance for successful outcomes in such patients.- - - - - - - - - - ranking = 2keywords = liver (Clic here for more details about this article) |
7/92. Hemophagocytic syndrome in pregnancy.BACKGROUND: Hemophagocytic syndrome is characterized by nonmalignant histiocytes that undergo uncontrolled phagocytosis of normal hematopoietic cells. Clinical severity ranges from complete recovery to rapid deterioration and death. CASE: thrombocytopenia was discovered upon routine initial prenatal evaluation of a 24-year-old, gravida 2, para 1, at 29 weeks' gestation with a history of necrotizing lymphadenitis. Cytopenia and elevated transaminases developed, followed by hyperpyrexia. The patient delivered and her postpartum course was complicated by coagulopathy, multiorgan failure, and death. bone marrow biopsy confirmed hemophagocytic syndrome. CONCLUSION: early diagnosis of hemophagocytic syndrome during pregnancy might be helped by recognizing symptoms and signs, including a history of necrotizing lymphadenitis, and obtaining a bone marrow biopsy.- - - - - - - - - - ranking = 1keywords = liver (Clic here for more details about this article) |
8/92. Use of radiation therapy in posttransplant lymphoproliferative disorder (PTLD) after liver transplantation.Posttransplant lymphoproliferative disorder (PTLD) is a common and life-threatening complication of immunosuppression used to prevent rejection of solid organ and bone marrow transplants. There is no standardized treatment algorithm, but numerous management strategies are available. We describe a patient who developed a solitary lymphoproliferative lesion in the porta hepatis 9 months after orthotopic liver transplant. Following reduction in immunosuppression with no response, she was treated with involved field radiotherapy utilizing CT-based treatment planning. A partial radiographic response was obtained, and she has not developed disease in the engrafted liver or systemically. Based on the present case report, involved field radiotherapy seems to be a reasonable treatment option for patients with localized PTLD. Int. J. Cancer (Radiat. Oncol. Invest.) 90:104-109, 2000.- - - - - - - - - - ranking = 6keywords = liver (Clic here for more details about this article) |
9/92. Hemophagocytic syndrome following an Epstein-Barr virus infection: a case report and literature review.Hemophagocytosis is an uncommon disorder characterized by proliferation of histiocytes that actively engulf other hematopoietic cells causing cytopenia. Reactive or secondary hemophagocytosis is very rare in healthy adults in the US. Various infectious, as well as neoplastic and immunologic etiologies of reactive hemophagocytosis have been reported. It is a non-malignant, reactive disorder characterized by hemophagocytosis in the bone marrow and reticuloendothelial system (RES) resulting in pancytopenia, fever, hepatic dysfunction, and disseminated intravascular coagulation (DIC). No consensus exists in the literature regarding optimal treatment of virus-associated hemophagocytic syndrome (VAHS). We report a case of VAHS in a previously healthy immunocompetent male and review the diagnosis and management of this rare disorder.- - - - - - - - - - ranking = 0.29217588011858keywords = hepatic (Clic here for more details about this article) |
10/92. 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 = 3keywords = liver (Clic here for more details about this article) |
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