Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound   Target Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound

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Query: UMLS:C0021051 (immunodeficiency)
71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This report describes two cases of Kaposi's sarcoma-associated herpesvirus/human herpesvirus-8 (KSHV/HHV8)-associated lymphomas that primarily involved the large bowel and that secondarily caused malignant effusions. Involvement of the gastrointestinal tract is of interest because epidemiologic evidence suggests that KSHV/HHV-8 may be transmitted via the fecal-oral route, and KSHV/HHV8 DNA has been detected in rectal samples from HIV-positive patients. This report describes two HIV-positive men who developed primary KSHV/ HHV8-associated lymphomas of the bowel. Despite similar morphology and immunophenotype, these cases differ from most KSHV/HHV8-associated primary effusion lymphomas, which present with malignant effusions in the absence of a solid tumor mass. The spectrum of KSHV/HHV8-associated lymphomas is expanded to include a subset of primary bowel lymphomas in individuals infected with human immunodeficiency virus.
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PMID:Kaposi's sarcoma-associated herpesvirus and human herpesvirus 8 (KSHV/HHV8)-associated lymphoma of the bowel. Report of two cases in HIV-positive men with secondary effusion lymphomas. 1043 72

Human herpes virus 8 (HHV-8; or KSHV, Kaposi's sarcoma-associated herpes virus) is a gamma herpes virus with sequence homology to Epstein-Barr virus (EBV). It was first isolated from Kaposi's sarcoma tumor cells and subsequently from tumor cells and peripheral blood mononuclear cells from patients with primary effusion lymphomas (PEL; or body cavity-based lymphomas). PEL has been recognized as an individual nosologic entity based on its distinctive biological-pathological features and its consistent infection with HHV-8 (commonly, but not always co-infected with EBV), occurring predominantly in human immunodeficiency virus (HIV)-infected patients but occasionally also in HIV-negative cases. Whether HHV-8 sequences can be found also in non-hematopoietic tumor cells other than Kaposi's sarcoma and in malignant hematopoietic malignancies other than PEL, has been the focus of the present studies. We examined the presence of HHV-8 sequences by polymerase chain reaction (PCR) using (1) a panel of 133 human cell lines established from a large variety of solid tumors; (2) a spectrum of 114 hematopoietic cell lines derived from the different cell lineages including 50 B cell leukemia/lymphoma-derived cell lines and seven cell lines established from patients with PEL. Besides the seven PEL cell lines, 46 cell lines that were derived from malignant pleural effusion or ascitic fluid material (25 non-hematopoietic and 21 hematopoietic cell lines) were examined. Except for the seven PEL cell lines that were strongly HHV-8+ in the PCR, all solid tumor cell lines and all hematopoietic cell lines scored consistently negative for the presence of HHV-8 sequences. These results confirm the absolute specificity of HHV-8 infection (within the hematopoietic malignancies) for PEL. PEL cell lines represent useful tools for the analysis of the biology of this neoplasm and of the pathogenetic role of the virus in the disease development.
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PMID:HHV-8 infection is specific for cell lines derived from primary effusion (body cavity-based) lymphomas. 982 57

Human herpesvirus 8 (HHV-8), or Kaposi sarcoma-associated herpesvirus, is a gamma herpesvirus first detected in a specimen of Kaposi sarcoma from a human immunodeficiency virus (HIV)-positive patient. Human herpesvirus 8 is also found in an unusual clinicopathologic form of body cavity-based B-cell lymphoma, which has been named primary effusion lymphoma (PEL) and occurs primarily in HIV-positive patients. PEL is characterized by the formation of lymphomatous effusions, without obvious lymphadenopathy, tumor masses, or bone marrow involvement. Only a few cases of PEL in HIV-seronegative patients have been reported. We describe a case of an HHV-8-associated lymphoma, with ascites, pleural effusion, and axillary lymphadenopathy in an HIV-negative patient. The patient was a 68-year-old Jewish man of North African extraction, with a previous history of coronary bypass surgery and multiple blood transfusions. The pleural fluid contained large atypical lymphoid cells and was suggestive of lymphoma but could not provide a conclusive diagnosis of PEL. The lymph node contained groups of large anaplastic lymphoid cells. Polymerase chain reaction for HHV-8 performed on the lymph node specimen was positive, establishing the diagnosis of PEL. Polymerase chain reaction for Epstein-Barr virus was negative. Results of a gallium scan were normal. The patient did not respond to combination chemotherapy with cyclophosphamide, doxorubicin, vincristine sulfate, and prednisone and progressively developed, massive intra-abdominal solid tumor formation. To our knowledge, this is the first report of a case of PEL that demonstrates peripheral lymph node involvement at diagnosis and the first report of PEL in an Israeli patient.
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PMID:Early peripheral lymph node involvement of human herpesvirus 8-associated, body cavity-based lymphoma in a human immunodeficiency virus-negative patient. 1078 62

Primary effusion lymphoma (PEL) is a B-cell neoplasm characterized by infection of the tumor clone by human herpesvirus type-8/Kaposi's sarcoma-associated herpesvirus (HHV-8/KSHV) and by liquid growth in fluid-filled body spaces. During its entire clinical course, the lymphoma tends to remain localized to the serous body cavities with no formation of solid tumor masses. The epidemiology of PEL points to a close link with underlying immunodeficiency of the host, as most cases develop in individuals severely immunocompromised because of preexisting acquired immunodeficiency syndrome. The histogenesis and pathogenesis of PEL have been clarified to a sizeable extent by intensive investigations performed since the disease recognition in 1995. PEL is composed of postgerminal center B cells, which bridge immunoblastic and anaplastic features and typically display a non-B, non-T phenotype consistent with late stages of B-cell differentiation. HHV-8/KSHV is thought to play a major role in PEL pathogenesis via expression of several viral latent genes, which have the potential to affect B-cell growth. Other factors involved in PEL pathogenesis include deregulation of cytokine and growth factor autocrine loops, molecular alterations of the tumor DNA, cell cycle abnormalities, stimulation and selection by antigen, and infection by Epstein-Barr virus, which occurs in 70% of PEL cases. In the years since the disease discovery, the distinctiveness of the biological and clinicopathological features of PEL has prompted its recognition as an independent lymphoma category by the World Health Organization classification system of hematologic neoplasms.
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PMID:Primary effusion lymphoma: a liquid phase lymphoma of fluid-filled body cavities. 1103 42

Allogeneic bone marrow transplantation (BMT) is currently restricted to hematological malignancies because of a lack of antitumor activity against solid cancers. We have tested a novel treatment strategy to stimulate specific antitumor activity against a solid tumor after BMT by vaccination with irradiated tumor cells engineered to secrete granulocyte-macrophage colony-stimulating factor (GM-CSF). Using the B16 melanoma model, we found that vaccination elicited potent antitumor activity in recipients of syngeneic BMT in a time-dependent fashion, and that immune reconstitution was critical for the development of antitumor activity. Vaccination did not stimulate antitumor immunity after allogeneic BMT because of the post-BMT immunodeficiency associated with graft-versus-host disease (GVHD). Remarkably, vaccination was effective in stimulating potent and long-lasting antitumor activity in recipients of T-cell-depleted (TCD) allogeneic bone marrow. Recipients of TCD bone marrow who showed significant immune reconstitution by 6 weeks after BMT developed B16-specific T-cell-cytotoxic, proliferative, and cytokine responses as a function of vaccination. T cells derived from donor stem cells were, therefore, able to recognize tumor antigens, although they remained tolerant to host histocompatibility antigens. These results demonstrate that GM-CSF-based tumor cell vaccines after allogeneic TCD BMT can stimulate potent antitumor effects without the induction of GVHD, and this strategy has important implications for the treatment of patients with solid malignancies.
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PMID:Tumor cell vaccine elicits potent antitumor immunity after allogeneic T-cell-depleted bone marrow transplantation. 1119 55

Epstein-Barr virus-associated lymphoproliferative disease (EBV-LPD) is a serious disorder seen in various states of immunodeficiency, often with a fatal outcome. In this article, a patient with EBV-lymphoma after autologous stem cell rescue for treatment of a nonhematologic solid tumor is described. The child, a 4-year-old boy, had unilateral retinoblastoma with metastatic spread to the central nervous system. He had previously received both local tumor bed and craniospinal radiation therapy together with intensive myeloablative alkylator chemotherapy before autologous stem cell rescue. Histologically confirmed lymphoma with evidence of active EBV proliferation developed within cervical lymph nodes 3 weeks after his first autologous stem cell rescue. A complete clinical remission of the lymphadenopathy was obtained after infusions of rituximab (an anti-CD20 monoclonal antibody), acyclovir, and high-titer anticytomegalovirus immunoglobulin. The patient died approximately 6 months later of persistent and progressive retinoblastoma without any clinical evidence of lymphoma. It is concluded that EBV-LPD should be included in the differential diagnosis in patients in whom lymphadenopathy develops after autologous stem cell rescue.
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PMID:Epstein-Barr virus-associated lymphoma in a child undergoing an autologous stem cell rescue. 1199 94

Primary effusion lymphoma is a distinct clinicopathologic entity usually characterized by presentation as a lymphomatous body cavity effusion in the absence of a solid tumor mass or dissemination during its clinical course. This lymphoma is typically present in human immunodeficiency virus (HIV)-infected patients and frequently associated with Kaposi's sarcoma-associated herpesvirus/human herpesvirus 8 (KSHV/HHV8) viral sequences. Here we report a rare case of KSHV/HHV8-associated primary effusion lymphoma with secondary involvement of the small bowel as an obstructive tumor mass in an HIV-infected man. The solid small bowel lymphoma demonstrated essentially identical morphology, immunophenotype, KSHV/HHV8 viral status, and immunoglobulin light chain rearrangements to the pleural cavity-based primary effusion lymphoma in the same patient.
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PMID:Primary effusion lymphoma with subsequent development of a small bowel mass in an HIV-seropositive patient: a case report and literature review. 1236 52

A 51-year-old man without human immunodeficiency virus, hepatitis B virus or hepatitis C virus was admitted with left scrotum swelling and hydrocele. The cytological finding of fluid in the left scrotum revealed malignant lymphoma, and the immunophenotypic analysis and monoclonal rearrangement of immunoglobulin heavy chain demonstrated B-cell lymphoma. However, no solid tumor of lymphoma was identified in the specimen following a left orchiectomy, or in any other body site and genomic human herpes virus-8 and Epstein-Barr virus were not detected in the lymphoma cells. So we interpreted this as a primary effusion lymphoma without any ethological viral infection. Subsequently, he underwent chemo-radiation therapy and has remained in remission.
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PMID:Primary effusion lymphoma of the left scrotum. 1272 25

Primary effusion lymphomas (PELs) are specifically associated with Kaposi's sarcoma-associated herpesvirus (KSHV) infection and most frequently occur in human immunodeficiency virus (HIV)-positive individuals as lymphomatous effusions in the serous cavities without a detectable solid tumor mass. Most PELs have concomitant Epstein-Barr virus (EBV) infection, suggesting that EBV is an important pathogenetic cofactor, although other as yet unidentified cofactors, such as cellular genetic alterations, are also likely to play a role. Lymphomatous effusions that lack KSHV also occur; these are frequently EBV associated in the setting of HIV infection. Here we used gene expression profile analysis to determine the viral impact on cellular gene expression and the pathogenesis of these lymphomatous effusions. Our results show that many genes, including cell cycle and signal transduction regulators, are differentially expressed between KSHV-positive PELs and KSHV-negative lymphomatous effusions and also between KSHV-positive, EBV-positive and KSHV-positive, EBV-negative PELs. Our results confirm that KSHV plays an important role in the pathogenesis of PELs, as its presence selects for a very distinct cellular gene expression category and a clearly different lymphoma type. Within the KSHV-positive PELs, the effect of EBV is more subtle but nevertheless clear.
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PMID:Distinct subsets of primary effusion lymphoma can be identified based on their cellular gene expression profile and viral association. 1561 51

Primary effusion lymphoma (PEL) is an uncommon large cell lymphoma, usually seen in human immunodeficiency virus (HIV)-infected patients. PEL is characterized by various clinical, histomorphologic, and immunophenotypical features, and is associated with the human herpes virus 8 (HHV-8). PEL may present as either a body cavity-based lymphomatous effusion or a solid tumor mass. Most so-called "solid PEL" usually have an extranodal location; exceptionally rarely, they occur in lymph nodes. The majority of PEL consist of malignant cells of B-cell genotype; seldom they are of T-cell origin. We report a rare case of HHV-8-associated "solid PEL" of T-cell type in a 41-year-old HIV-seropositive man with a concomitant peritoneal effusion. The T-cell lymphoma was diagnosed on the basis of morphologic, immunophenotypic, and molecular findings of a lymph node biopsy. The tumor cells strongly expressed CD45R0, CD7, CD43, MUM1/IRF4, CD30, HHV-8, and EBER, and demonstrated a clonal rearrangement of T-cell receptor-gamma chain gene. The following case provides another example of a lymph node-based "solid" PEL, demonstrating the variety within the spectrum of HHV-8-associated lymphoma.
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PMID:HHV-8-associated T-cell lymphoma in a lymph node with concurrent peritoneal effusion in an HIV-positive man. 1583 89


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