Cases reported "Herpesviridae Infections"

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1/128. lymphomatoid granulomatosis following autologous stem cell transplantation.

    lymphomatoid granulomatosis (LYG) is a rare angio-destructive lymphoproliferative disorder (LPD) of uncertain etiology, with prominent pulmonary involvement. Recent studies indicate that LYG is an Epstein-Barr virus (EBV)-associated B cell LPD with large numbers of background reactive T lymphocytes (T cell-rich B cell lymphoma). Although the disease frequently, but not exclusively, occurs in various immunodeficiency states, it has not been reported in association with the transient immunosuppression following autologous bone marrow/peripheral stem cell transplantation (ABM/PSCT). We describe a patient who developed lymphomatoid granulomatosis of the lung approximately 2 weeks after high-dose chemotherapy and autologous peripheral stem cell transplantation for multiple myeloma. Although molecular studies showed no evidence of EBV genome in the biopsy material, the serologic profile with high IgM titers was suggestive of primary EBV infection. Complete radiologic remission occurred following reconstitution of the patient's immune response after a 2-week course of ganciclovir treatment. Despite the apparently low frequency of LPD (both LYG and EBV-associated post-transplant lymphoma) in the ABMT setting, we believe that it should be considered in the differential diagnosis of patients whose clinical course following ABMT is complicated by fevers, in the absence of an identifiable infectious process.
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ranking = 1
keywords = lymphoma
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2/128. Primary ocular Epstein-Barr virus-associated non-Hodgkin's lymphoma in a patient with AIDS: a clinicopathologic report.

    OBJECTIVE: To report an unusual case of chronic multifocal chorioretinitis with vitritis in a patient with acquired immunodeficiency syndrome (AIDS) that was resistant to antiviral and antitoxoplasmic medication and required a retinal biopsy for definitive diagnosis. methods: Vitreous biopsy, pars plana vitrectomy, and retinal biopsy were performed. The vitreous biopsy material was sent for bacterial, fungal, and viral culture, and the vitreous cassette was sent for cytology. The retinal biopsy material was divided and sent for polymerase chain reaction testing for toxoplasmosis and virology and pathologic tissue analysis. RESULTS: Vitreous cytology showed a mixed population of lymphocytes and histiocytes, but all other microbiologic and virologic studies were negative. Tissue analysis revealed an infiltrate of atypical mononuclear cells extending from the inner limiting membrane through the outer plexiform layer characteristic of a B cell, non-Hodgkin's lymphoma of the central nervous system (NHL-CNS). in situ hybridization for the Epstein-Barr virus (EBV) was positive. An extensive systemic evaluation did not show evidence of extraocular tumor. CONCLUSION: Although rare, primary ocular NHL-CNS can be seen in patients with AIDS, and its clinical presentation often closely resembles other disorders. To our knowledge, this case represents the first ocular NHL in which EBV is shown to be associated.
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ranking = 1.6666666666667
keywords = lymphoma
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3/128. Herpesvirus 8 inclusions in primary effusion lymphoma: report of a unique case with T-cell phenotype.

    We describe a case of primary effusion lymphoma with T-cell phenotype, mixed genotype, and intranuclear herpesvirus inclusions visible with the light microscope. cells were studied by immunohistochemical analysis, in situ hybridization, immunoglobulin and T-cell receptor gene rearrangement, and polymerase chain reaction. Primary effusion lymphoma cells with T-cell phenotype revealed herpesvirus 8 inclusions predominantly seen in apoptotic cells, suggesting that productive viral infection is associated with cell death. Clinical features were typical of primary effusion lymphoma. Cytologic, molecular genetic, and phenotypic features demonstrated a unique variant of primary effusion lymphoma.
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ranking = 2.6666666666667
keywords = lymphoma
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4/128. Epstein-Barr virus related hemophagocytic syndrome in a T-cell rich B-cell lymphoma.

    We report the case of a 30-year-old woman who presented with an EBV related hemophagocytic syndrome. After a few months she developed a T-cell rich B-cell non-Hodgkin's lymphoma with liver involvement. Serological data demonstrated a reactivation of the EBV infection. Tumor progression with liver involvement occurred during treatment with conventional chemotherapy. Tumor reduction and disappearance of all masses was seen after starting high-dose sequential chemotherapy, followed by an autologous peripheral blood progenitor transplantation LMP-1 could be amplified in the tumor material by PCR technology, but no LMP-1 expression could be found in the few malignant B-cells with Reed-Sternberg morphology. sequence analysis of the carboxy terminal of the LMP-1 region revealed the naturally occurring 30 bp deletion variant of the LMP-1 with multiple point mutations within the NF kb region. Since LMP-1 was not expressed in the malignant tumor cells, no evidence could be found, that EBV participated in the tumorigenesis of this case.
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ranking = 1.6666666666667
keywords = lymphoma
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5/128. association of body cavity-based lymphoma and human herpesvirus 8 in an hiv-seronegative male. Report of a case with immunocytochemical and molecular studies.

    BACKGROUND: Recently lymphomas arising primarily in serosal surfaces have been found in patients with advanced acquired immunodeficiency syndrome (AIDS), but they very rarely seem to occur in human immunodeficiency virus (hiv)-negative patients. Studies on a subset of these lymphomas suggested that they represent a distinct entity associated with Kaposi's sarcoma-associated herpesvirus or human herpesvirus 8 (HHV-8). CASE: An 83-year-old, hiv-negative male was admitted to the hospital with a massive pleural effusion. Abdominal and chest computed tomographic scanning was normal. Cytologic analysis of the pleural effusion revealed a large cell, non-Hodgkin's lymphoma. polymerase chain reaction analyses on genomic dna from the pleural effusion demonstrated the presence of HHV-8 sequences in the absence of Epstein-Barr virus. CONCLUSION: It is possible and advantageous to diagnose body cavity-based lymphoma with a combination of cytologic, immunocytochemical and molecular studies of the pleural effusion in conjunction with clinical and radiographic information.
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ranking = 2.6666666666667
keywords = lymphoma
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6/128. Epstein-Barr virus-associated intravascular lymphomatosis within Kaposi's sarcoma in an AIDS patient.

    Intravascular lymphomatosis (IL) is an unusual neoplasm characterized by multifocal proliferation of lymphoma cells exclusively within the blood vessels. We report here a patient with acquired immunodeficiency syndrome (AIDS) and disseminated Kaposi's sarcoma. A 233-bp amplification product of HHV-8 was detected in the dna extracted from specimens of Kaposi's sarcoma at different sites by polymerase chain reaction (PCR). At autopsy, the vessels within the Kaposi's sarcoma were dilated and filled with atypical large mononuclear cells. No such feature was seen in the vessels of non-Kaposi's sarcomatous regions. Immunohistochemically, the spindle cells of Kaposi's sarcoma were positive for CD31 (endothelial cell marker). The intravascular tumor cells were positive for CD45 (leukocyte common antigen) but negative for others, including chloroacetate esterase, CD45-RO (UCHL-1, Pan-T), CD3, CD43, CD20 (L26, Pan-B), CD30 (Ki-1), immunoglobulin heavy chains and light chains, CD56 (natural killer cell antigen), and CD31. Monoclonal rearrangement of immunoglobulin heavy chain gene was detected in the dna extracts from fresh tissue of Kaposi's sarcoma by PCR, which indicated that the lymphoma cells within the Kaposi's sarcoma were of monoclonal B cell origin. in situ hybridization revealed that EBER-1 transcripts were present in the lymphoma cells of IL but not in the spindle cells of Kaposi's sarcoma. To the authors' best knowledge, this is the first instance of IL in an AIDS patient with direct evidence of EBV association.
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ranking = 2.6666666666667
keywords = lymphoma
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7/128. Post transplant CD8 gammadelta T-cell lymphoma associated with human herpes virus-6 infection.

    Gammadelta T-cell lymphoma is a rare T-cell lymphoproliferative disorder that has been reported in both immunocompetent and immunocompromised persons. This report describes a forty eight year old patient who developed gammadelta T-cell lymphoma four years after undergoing living-related kidney transplantation. The lymphoma expressed CD2, CD3, CD7, CD8 and CD56, and the gammadelta T-cell receptor and did not express CD5, CD4 and the alphabeta T-cell receptor. In addition, HHV-6 was cultured from the patient's bone marrow, marking the first time that this virus has been associated with gammadelta T-cell lymphoma. Since all patients with gammadelta T-cell lymphoma described to date have responded poorly to standard combination chemotherapies, the patient was treated with the purine analogue 2-chlorodeoxyadenosine. While he responded transiently to treatment, long term remission was not achieved indicating that additional therapeutic approches still need to be developed, for the management of this disorder.
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ranking = 3
keywords = lymphoma
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8/128. Monoclonal Epstein-Barr virus-related lymphoproliferative disorder following adult acute lymphoblastic leukaemia.

    A 31-year-old patient in remission of acute lymphoblastic leukaemia (ALL), receiving oral maintenance chemotherapy (6-mercaptopurine, methotrexate (MTX), cyclophosphamide), developed a monoclonal, Epstein-Barr virus (EBV)-related lymphoproliferative disorder (LPD). Treatment consisted of excisional biopsy and the discontinuation of maintenance chemotherapy. To our knowledge, this is the first such report in an adult. The histological similarity to previous reports of 'lymphomatoid granulomatosis' following paediatric ALL suggests that they are the same disease. MTX may play a central role in the development of LPD in this setting. Although it is a rare complication of ALL, EBV-related LPD should be considered in patients who develop lymphadenopathy.
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keywords = lymphoma
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9/128. Clinicopathological findings of virus-associated hemophagocytic syndrome in bone marrow: association with Epstein-Barr virus and apoptosis.

    Non-neoplastic hemophagocytic syndrome (HPS), also called virus-associated hemophagocytic syndrome (VAHS), has been thought to be a distinct clinical entity. A spontaneous recovery is common, but the prognosis of Epstein-Barr virus (EBV)-associated VAHS is poor. However, the role of EBV has yet to be clearly elucidated. A retrospective study of the bone marrow of 30 cases, in which the diagnosis of non-neoplastic VAHS was clinicopathologically confirmed, was performed. We were unable to histologically confirm the presence of neoplastic lesions, especially lymphoma cell infiltration. Ten of the patients were children (aged less than 15 years) and young adults (aged under 20 years; median age, 10 years). Twenty patients were adults (aged over 21 years; median age, 48 years). Twelve of these patients died, while 18 showed a spontaneous recovery. We performed immunological staining and in situ hybridization (ISH) for EBV. To clarify the presence of apoptosis, an in situ apoptosis detection (tunnel) method was used. In situ hydridization showed an EBV-presence in 16 of the 30 patients. In addition, the EBV-presence was confined in the lymphocytes, especially T lymphocytes in double stainings. The number of EBV-infected cells varied; however, the EBV presence was associated with ages. Nine of the 10 children and young adults showed an EBV-presence, while EBV was detected in seven of the 20 adults. Especially in 10 patients aged over 49 years, no EBV was detected. According to the in situ apoptosis detection, apoptotic cells were increased in number and considered to be lymphoid cells, but not myeloid or histiocytic cells. Some apoptotic cells were phagocyted with histiocytes. Histologically, apoptosis may be one of the factors that induced phagocytosis.
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ranking = 0.33333333333333
keywords = lymphoma
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10/128. Development of EBV-positive T-cell lymphoma following infection of peripheral blood T cells with EBV.

    Chronic active Epstein-Barr virus (EBV) infection is manifested clinically by the persistence of infectious mononucleosis-like symptoms or its complications for a prolonged period ranging from one to several years. This syndrome may include severe disease manifestations and can be fatal. The role of EBV in the pathogenesis of chronic active EBV infection has been unclear. We investigated two Japanese patients with severe chronic active EBV infection who subsequently developed EBV-positive T-cell lymphoma. We found that the patients had evidence of EBV infection in the peripheral blood CD4 T-cells 19 and 3 months, respectively, before the T-cell lymphoma was diagnosed. The lymphomas were infected with monoclonal EBV and expressed the EBV latency genes EBNA-1, LMP-1, and LMP-2A, a virus latency pattern referred to as latency II. Genetic studies showed that the virus detected in the T-cell lymphoma was indistinguishable from the virus in the peripheral blood CD4 T-cells. These studies support an important pathogenetic role of T-cell infection with EBV in chronic active EBV infection and in the EBV-positive T-cell lymphoma that followed.
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ranking = 3
keywords = lymphoma
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