Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0079731 (B-cell lymphoma)
 16,671 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Kaposi's sarcoma (KS) is a vascular tumor predominantly found in the immunosuppressed. Epidemiologic studies suggest that an infective agent is the etiologic culprit. Kaposi's sarcoma-associated herpesvirus (KSHV), or human herpesvirus-8 (HHV-8), is a gamma human herpesvirus present in all epidemiologic forms of KS and also in a rare type of a B cell lymphoma, primary effusion lymphoma (PEL). In addition, this virus is present in most biopsies from human immunodeficiency virus (HIV)-associated multicentric Castleman's disease (MCD). MCD is a lymphoproliferative disorder with, like KS, a prominent microvasculature. The genome of KSHV contains the expected open reading frames (ORFs) encoding for enzymes and viral structural proteins found in other herpesviruses, but it also contains an unprecedented number of ORFs pirated during viral evolution from cellular genes. These include proteins that may alter cellular growth (e.g., Bcl-2 and cyclin homologs), induce angiogenesis (e.g., chemokine, chemokine receptor, and cytokine homologs), and regulate antiviral immunity (e.g., CD21 and interferon regulatory factor homologs). No ORF with sequence similarity to the Epstein-Barr nuclear antigens (EBNAs) and latent membrane proteins (LMPs) of Epstein-Barr virus (EBV) is present, but proteins analogous to these in structure and in latent expression are found [e.g., ORF 73 encoding for KSHV latent nuclear antigen (LNA-1) and K12 encoding for a possible latent membrane protein]. Current serologic assays confirm the strong association of infection with KSHV and risk of KS development. The mechanism of how this new virus may trigger the precipitation of KS is still unclear.
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PMID:Kaposi's sarcoma-associated herpesvirus. 970 7

Human herpesvirus 8 (HHV-8) has been identified as the causative agent for all forms of Kaposi's sarcoma and is also associated with the development of body cavity-based B-cell lymphomas and multicentric Castleman's disease. HHV-8 genomes are now classified into five major subtypes (A-E) that reflect sequence heterogeneity in the highly variable open reading frame (ORF) K1. To identify HHV-8 subtypes associated with different forms of Kaposi's sarcoma, we compared the ORF 26 and ORF-K1 gene sequences from South African patients with the prototype strains of the major subtypes, as well as published sequences from other African strains. DNA prepared from Kaposi's sarcoma biopsies and/or peripheral blood lymphocytes were available from 14 patients with postrenal transplant (iatrogenic) Kaposi's sarcoma, six patients with the African endemic form, and one patient with AIDS-related body cavity-based B-cell lymphoma. We identified a B2 subtype in six patients, four of whom also had a novel B5 type ORF 26 polymorphism. Two patients had B2 type patterns for both the ORF 26 and ORF-K1 genes. The ORF-K1 subtype A5 was identified in samples from three patients with a B3/C2 type polymorphism in the ORF 26 gene. A novel ORF-K1 B variant strain was identified in a patient with African endemic Kaposi's sarcoma, who also had a B3/C2 class ORF 26 pattern. In 58.3% of iatrogenic Kaposi's sarcoma patients, a B5-type ORF 26 gene sequence pattern was identified. No association was found among particular subtypes, geographical origin of patients, or clinical presentation.
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PMID:HHV-8 subtypes in South Africa: identification of a case suggesting a novel B variant. 1178 33

Human herpesvirus 8 (HHV-8), also known as Kaposi's sarcoma-associated herpesvirus (KSHV), discovered in 1994, is a human rhadinovirus (gamma-2 herpesvirus). Unlike other human herpesviruses (herpes simplex virus, Epstein-Barr virus, varicella-zoster virus, cytomegalovirus, HHV-6, and HHV-7), it is not widespread in the general population and has many unique proteins. HHV-8 is strongly associated with all subtypes of Kaposi's sarcoma (KS), multicentric Castleman's disease, and a rare form of B-cell lymphoma, primary effusion lymphoma. In addition, HHV-8 DNA sequences have been found in association with other diseases, but the role of the virus in these diseases is largely unconfirmed and remains controversial. The seroprevalence of HHV-8, based on detection of latent and lytic proteins, is 2 to 5% in healthy donors except in certain geographic areas where the virus is endemic, 80 to 95% in classic KS patients, and 40 to 50% in HIV-1 patients without KS. This virus can be transmitted both sexually and through body fluids (e.g., saliva and blood). HHV-8 is a transforming virus, as evidenced by its presence in human malignancies, by the in vitro transforming properties of several of its viral genes, and by its ability to transform some primary cells in culture. It is not, however, sufficient for transformation, and other cofactors such as immunosuppressive cytokines are involved in the development of HHV-8-associated malignancies. In this article, we review the biology, molecular virology, epidemiology, transmission, detection methods, pathogenesis, and antiviral therapy of this newly discovered human herpesvirus.
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PMID:Spectrum of Kaposi's sarcoma-associated herpesvirus, or human herpesvirus 8, diseases. 1209 51

A rare simultaneous occurrence of multicentric Castleman's disease, non-Hodgkin's lymphoma, and Kaposi's sarcoma was diagnosed in a 70-year-old man who presented with fever, polyarthralgia, weight loss, vascular purpura, anemia, generalized lymphadenopathy, and hepatosplenomegaly. He had no risk of HIV infection and serological tests for HIV were negative twice, but a low number of T-cells and a reversed CD4/CD8 ratio were observed. During hospitalization, he developed Kaposi's sarcoma at the right sole. Lymph node biopsies revealed multicentric Castleman's disease together with a large B-cell lymphoma, which showed monotypic IgM-lambda lymphocytes. To our knowledge, this is the first report in which systemic manifestations of all three diseases occurred simultaneously prior to any specific treatment. The altered immune status and human herpesvirus-8 infection might have played a role in the pathogenesis of this occurrence.
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PMID:Multicentric Castleman's disease, non-Hodgkin's lymphoma, and Kaposi's sarcoma: a rare simultaneous occurrence. 1240 98

Kaposi's sarcoma-associated herpesvirus, the viral agent of Kaposi's sarcoma, is associated with two lymphoproliferative disorders: primary effusion lymphoma and multicentric Castleman's disease. To identify other lymphoproliferative conditions linked with Kaposi's sarcoma-associated herpesvirus, we studied non-Hodgkin's lymphomas arising in individuals with AIDS-associated Kaposi's sarcoma. Formalin-fixed tissues from 24 such lymphomas were examined. As expected, two primary effusion lymphomas were Kaposi's sarcoma-associated herpesvirus-positive, with immunohistochemistry demonstrating the Kaposi's sarcoma-associated herpesvirus latency-associated nuclear antigen in the nuclei of all neoplastic cells. Additionally, three of seven evaluable cases of the immunoblastic variant of diffuse large B-cell lymphoma (immunoblastic lymphoma) showed similar latency-associated nuclear antigen staining. These Kaposi's sarcoma-associated herpesvirus-positive immunoblastic lymphomas resembled primary effusion lymphoma histologically but were not known to involve body cavities (sites included lymph nodes, soft tissues of the neck, and spleen). Notably, 5-20% of the neoplastic cells in the Kaposi's sarcoma-associated herpesvirus-positive immunoblastic lymphomas also showed cytoplasmic staining for viral interleukin-6, a biologically active cytokine homologue found in primary effusion lymphoma. We conclude that Kaposi's sarcoma-associated herpesvirus is present in some immunoblastic lymphomas in persons with AIDS-associated Kaposi's sarcoma.
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PMID:Immunoblastic lymphoma in persons with AIDS-associated Kaposi's sarcoma: a role for Kaposi's sarcoma-associated herpesvirus. 1274 48

There is currently no consensus on the best treatment for unresectable hyaline-vascular variant or for multicentric Castleman's disease (MCD), because none of the reported regimens have consistently produced complete response or durable remission in the majority of patients In the present study, we report on the use of 2-CdA (2-chloro-deoxyadenosine) in three patients, two of them with MCD and one with unresectable hyaline-vascular type disease. Relapse-free survival of the responding patients was 24 and 20 months. Later, both patients evolved to non-Hodgkin's lymphoma (NHL) (diffuse large B-cell lymphoma and peripheral T-cell NHL, respectively). 2-CdA typically causes a long-lasting state of immunodeficiency and the profound influence of this drug on the immune system has raised questions concerning the emergence of secondary neoplasms after its use. Therefore, it is reasonable to conclude that: 1) 2-CdA can induce durable complete remission in MCD patients but unfortunately it cannot cure the disease; 2) the possibility that 2-CdA may accelerate the transformation of MCD to NHL cannot be ruled out.
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PMID:2-Chloro-deoxyadenosine induces durable complete remission in Castleman's disease but may accelerate its transformation to non-Hodgkin's lymphoma. 1469 Jan 66

Idiopathic plasmacytic lymphadenopathy (IPL) with polyclonal hyperimmunoglobulinemia is considered identical to multicentric Castleman's disease (MCD) reported in western countries. Clinically, both IPL and MCD are characterized by multicentric lymphadenopathy, prominent polyclonal hypergammaglobulinemia, elevated erythrocyte sedimentation rate, elevated serum interleukin-6 concentration, bone marrow plasmacytosis, and various abnormal laboratory data such as anemia and positive autoantibodies. However, IPL has a significantly better 5-year survival rate than that of MCD. Moreover, none of the present 16 cases developed Kaposi's sarcoma or B-cell lymphoma. Histologically, the interfollicular area contains a sheet of polytypic mature plasma cells in both IPL and MCD. In MCD, the majority of lymphoid follicles had hyaline-vascular germinal centers. However, lymphoid follicles of IPL usually exhibit a hyperplastic germinal center. Immunostaining also demonstrated a normal/reactive follicular dendritic cell network pattern in the germinal center of IPL. Moreover, there were no human herpes virus-8-positive cells detected by immunohistochemistry. The overall clinicopathologic and immunohistochemical findings of our 16 cases suggest that IPL is distinct from MCD reported in Western countries.
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PMID:Clinical implication of idiopathic plasmacytic lymphadenopathy with polyclonal hypergammaglobulinemia: a report of 16 cases. 1573 69

We report a case of BCL-6-positive B cell lymphoma with human herpesvirus 8 (HHV-8) infection. A human immunodeficiency virus-infected patient developed a diffuse large B cell lymphoma, which was found exclusively in the liver and spleen with the absence of lymphadenopathy and effusion in any body cavities. The lymphoma cells were composed of medium to large-sized cells positive for CD20, CD45, and BCL-6, and negative for epithelial cell membrane antigen, CD30, CD45RO, and CD138/syndecan-1, suggesting a germinal center B cell origin. The patient was serologically positive for HHV-8, and HHV-8 was detected in the liver biopsy tissue both by polymerase chain reaction and by immunohistochemistry for HHV-8-encoded latency-associated nuclear antigen. Other HHV-8-associated diseases, such as Kaposi's sarcoma, primary effusion lymphoma, or multicentric Castleman's disease were not detected in the patient. Chemotherapy was effective and reduced the size of the lymphoma dramatically. This is the first case report of a germinal center B cell-originating lymphoma with HHV-8 infection.
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PMID:BCL-6-positive human herpesvirus 8-associated solid lymphoma arising from liver and spleen as multiple nodular lesions. 1537 Feb 68

Lymphadenopathy, which may be associated with systemic symptoms, is frequently associated with rheumatoid arthritis (RA). Reactive non-neoplastic tissue comprises the majority of the lymph node lesions. However, several cohort studies have demonstrated that RA has an increased risk of non-Hodgkin's lymphomas (NHLs). Since the early 1990s, an atypical or malignant lymphoproliferative disorders (LPD) in patients immunosupressed with methtorexate (MTX) therapy for RA has been emphasized, namely MTX-associated LPDs. Epstein-Barr virus (EBV) has received attention in connection with the etiology of RA. The present review describes the clinicopathologic and immunohistochemical findings of reactive, atypical and malignant LPDs associated with RA along with the presence or absence of EBV in LPDs using the in situ hybridization (ISH) method. The majority of reactive lymph node lesions exhibit reactive follicular hyperplasia with interfollicular polyclonal plasmacytosis. Atypical LPDs rarely appears in RA patients. However, these cases occasionally pose difficult problems in the differential diagnosis from malignant lymphomas associated with RA or atypical and malignant LPDs showing RA-like clinicopathological findings. Clinicopathologically, three types of atypical LPDs have delineated, i.e. (i) resembling multicentric Castleman's disease (MCD); (ii) atypical paracortical hyperplasia with lymphoid follicles (APHLF) and; (iii) atypical lymphoplasmacytic immunoblastic proliferation. Malignant lymphoma associated with RA is characterized by; (i) predominance of elderly cases; (ii) usually female predominance, reflecting the sex ratio of RA; (iii) longstanding history of RA; (iv) relatively frequent advanced stage of disease; (v) majority of the patients had the B-cell phenotype; and (vi) an increased frequency of diffuse large B-cell lymphoma (DLBCL) in RA. It is unlikely that EBV is the causative agent of either reactive or atypical LPD. Among malignant lymphomas, EBV-associated lymphoma comprised only a small fraction of all NHLs in the general RA patient population.
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PMID:Benign, atypical and malignant lymphoproliferative disorders in rheumatoid arthritis patients. 1706 72

Angiofollicular lymphoid hyperplasia (Castleman's disease) is a lymphoproliferative process thought to be mediated by overexpression of II interleukin-6. Castleman's disease has two variants: Castleman's disease has two variants: Hyaline vascular type and plasma cell variant (multicentric Castleman's disease). The hyaline vascular type tends to be localized, and the plasma cell variant shows more systematic signs and carriers a worse clinical prognosis. Castleman's disease is associated with B-cell lymphoma, Kaposi sarcoma, Human herpes virus 8 (HHV-8), and Epstein-Barr virus. Castleman's disease have been described thrice post kidney transplant. In this report, we document the course of a renal recipient who developed the plasma cell variant of Castleman's disease at 16 months after failure of his allograft and return to dialysis. He displayed clinical resolution of this complication after graft nephrectomy. To our knowledge, this is the first case where the disease manifestations disappeared after graft removal. Our patient experienced chronic renal allograft rejection which may have driven all the systematic manifestations of multicentric castleman's disease and possibly reactivated a latent HHV-8 infection. In this case immunohistochemical testing for HHV-8 was not available to prove a role for this agent.
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PMID:Post renal transplant Castleman's disease resolved after graft nephrectomy: a case report. 1752 52


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