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

Both Epstein-Barr virus (EBV) types A and B are found in endemic Burkitt's lymphoma (BL) occurring in equatorial Africa. We studied 17 cases of Brazilian BL previously demonstrated to be EBV-positive to determine the EBV type as well as the presence of a characteristic 30 bp deletion within the 3' end of the latent membrane protein-1 (LMP-1) gene that may be important to the pathogenesis of several EBV-associated neoplasms. All cases in which the age was known were children. We found type A EBV in 13 of 14 (93%) evaluable cases, and type B in one case. The LMP-1 deletion was found in 12 of 15 (80%) evaluable cases, including the one case of type B EBV, and a similar high prevalence (59%) of the deletion was detected in EBV-positive normal and reactive lymphoid tissues from individuals from the same geographic region. The high proportion of cases associated with type A EBV suggests that immunodeficiency is not an important factor in the pathogenesis of Brazilian BL, in contrast to endemic African BL. The presence of the LMP-1 deletion in a high prevalence in the normal population in this region is unexplained.
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PMID:Genotyping of Epstein-Barr virus in Brazilian Burkitt's lymphoma and reactive lymphoid tissue. Type A with a high prevalence of deletions within the latent membrane protein gene. 854 4

In a previous study, we described a cell line (BRG-P) derived from a woman with Burkitt's lymphoma (BL) and acquired immunodeficiency syndrome that shared the same characteristic cytogenetic abnormalities as the patient's malignant cells. This cell line contained subclones that displayed an isotype switch from IgM to IgA1 and an accumulation of point mutations in the Vh region genes. Because these two features suggested an antigen-driven process, we began a search for the antigen responsible for the stimulation of the malignant B cells. Specifically, we hypothesized that because the patient's tumor had presented as a lymphomatous infiltration of the breast, the malignant B cells were recruited to this site because of the reactivity of their surface lg with breast tissue. A hybridoma (BRG-H) was obtained by fusing BRG-M cells (an IgM producing subclone of the BRG-P cell) with an appropriate cellular partner. The monoclonal antibody (BRG MoAb) produced by this hybridoma reacted strongly with two of five breast cancer cell lines and stained normal and malignant ductal epithelial cells on breast tissue sections. The antigen recognized by the BRG MoAb consisted of a single, minimally glycosylated polypeptide chain of 45 kD (p45). The BRG MoAb failed to react with a panel of human cell lines from different tissues, except for one cell line from a uterine cervical carcinoma. No reactivity was detected for a panel of exogenous antigens from various pathogens, including human immunodeficiency virus and self-antigens frequently recognized by polyspecific antibodies. Experiments were performed to investigate the functional consequences of the interaction of surface IgM with its specific ligand. Coculture of BRG-M cells with p45+, but not with p45-, breast cells caused apoptosis of BRG-M cells. The specificity of the interaction was shown by the observation that apoptosis was prevented by pretreatment of BRG-M cells with a monovalent F(ab') fragment of rabbit IgG antibody to human mu chains. Moreover, only BRG-M cells, but not other BL cells, underwent apoptosis after exposure to p45+ breast cells. The interaction between the CD40 molecule expressed by BRG-M cells and its specific ligand (CD40L) prevented p45-induced cell apoptosis. Because this interaction mimics that occurring in vivo between T and B cells during immune responses, our data suggest that various events contributed to the emergence of the BL, in this particular patient, including antigenic stimulation possibly assisted by T-cell help.
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PMID:Apoptosis of Burkitt's lymphoma cells induced by specific interaction of surface IgM with a self-antigen: implications for lymphomagenesis in acquired immunodeficiency syndrome. 869 8

A human immunodeficiency virus-negative male was successfully treated for two occurrences of Burkitt's lymphoma, 15 years apart. As consolidation of his second remission, he underwent high-dose chemotherapy with peripheral blood stem cell transplantation. In an effort to prove whether the second lymphoma was a relapse of the first or a second primary lymphoma, we obtained paraffin-embedded material from both lymphomas. DNA was extracted from this material and amplified by polymerase chain reaction (PCR) using consensus JH and VH region primers. Analysis of the PCR products, which mostly reflects VDJ joints, showed two sharp bands of different molecular size, proving the monoclonal nature of the lymphomas and suggesting that each had different Ig gene rearrangements. Sequencing of both PCR products showed a marked dissimilarity in nucleotide sequence in the clonally unique VDJ joint region, providing strong evidence for the separate cellular genesis of each lymphoma. These results suggest that late relapses of Burkitt's lymphoma should be examined for clonal distinctiveness. If the second lymphoma is distinct from the primary one, it might be treated as a primary lymphoma rather than as recurrent disease.
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PMID:A clonally distinct recurrence of Burkitt's lymphoma at 15 years. 869 60

Substantial evidence indicates that several common viruses are clearly or probable causal factors in the etiology of specific malignancies. These viruses either normally establish latency or can become persistent infections. Oncogenesis is probably linked to an enhanced level of viral activation in the infected host, reflecting heavy viral dose or compromised immune control. The major virus-malignancy systems include hepatitis B virus (HBV), hepatitis C virus (HCV), and hepatocellular carcinoma; human lymphotropic virus-type 1 (HTLV-1) and adult T-cell leukemia/lymphoma (ATL); Epstein-Barr virus (EBV) and endemic Burkitt's lymphoma, nasopharyngeal carcinoma, and Hodgkin's disease; and human papilloma virus (HPV) and cervical cancer. Of these, a vaccine is available only for HBV. These malignancies tend to occur in early to mid-life and account for a substantial amount of morbidity and person-years lost. They are also likely to occur as "opportunistic malignancies" among individuals infected with human immunodeficiency virus type-1, particularly among those who experience prolonged survival.
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PMID:Overview: viral agents and cancer. 874 95

Extensive studies have demonstrated that Epstein-Barr virus (EBV) is associated with Burkitt's lymphoma, immunodeficiency related lymphoma, Hodgkin's disease and T-cell lymphoma. In order to investigate whether an association exists between EBV and B-cell lymphoma in patients without overt immunodeficiency, we examined 127 B-cell lymphomas without overt immunodeficiency for expression of EBV-encoded small RNA (EBER-1) using RNA/RNA in situ hybridization. EBER-1 expression was found in nuclei of tumor cells in 8 of 127 cases (6.3%) of B-cell lymphomas. This result is similar to that of studies in Europe and the United States (about 5%). The low frequency of EBV in B-cell lymphomas suggests that EBV does not pay an important role in the pathogenesis of B-cell lymphomas without overt immunodeficiency.
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PMID:[A study of the association between EBV and B-cell lymphoma]. 876 30

A strong association was found to exist between patterns of lymphoid malignancies and socioeconomic status. B-cell lymphomas and T-acute lymphoblastic leukemia are much more prevalent in developing countries where the chances of acquiring infections especially at a younger age are high. B-cell precursor acute lymphatic leukemia, however, are much more prevalent in the Western world. Many infectious agents are associated with lymphatic malignancies. Epstein-Barr virus is involved in African Burkitt's lymphoma, human immunodeficiency virus-related Burkitt's lymphoma, lymphoproliferative syndrome post-transplantation, and Hodgkin's disease. Other infectious agents which may play a role in lymphoproliferative disorders are human immunodeficiency virus in acquired immune deficiency syndrome-associated lymphoma, human T-lymphotropic virus in adult T-cell lymphoma, Helicobacter pylori in mucosa-associated lymphoid tissue lymphoma, theileriosis in lymphoproliferative syndrome in cattle, Avian leukosis virus in chicken bursal lymphoma, and possibly a bacterial infection in immunoproliferative small intestine disease, potentially reversed by antibiotic therapy. The association between infectious agents and hematologic malignancies may be explained by the creation of large populations of activated cells followed by higher occurrences of 'genetic accidents'. This theory may be reinforced in at least some malignancies with the existence of viral proteins which either have complex relationships with key cellular gene products like p53 and Rb which have roles in cell cycle control, or share common motifs with bc1-2, therefore operating as anti-apoptotic elements. Whenever these genes are deranged, cell deoxysibonucleic acid repair or apoptosis are no longer possible, thereby creating a state of genome instability, increased acquisition of mistakes, and increased chances for malignant transformation.
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PMID:Infectious agents and environmental factors in lymphoid malignancies. 881 40

The differences between reactive and malignant processes are sometimes blurred. Homogeneity is no longer a requisite for the diagnosis of lymphoma, as witnessed in mucosa-associated lymphatic tissue lymphoma and T-cell-rich B cell lymphoma, which are composed of an admixture of neoplastic clonal B cells and reactive T cells which occasionally are very prominent in the histological picture. Infectious mononucleosis, anaplastic large cell lymphoma, composite lymphoma and Hodgkin's disease, all share many similarities and may actually represent a continuous spectrum of pathological conditions. Immunodeficiency states, whether primary or acquired, are commonly associated with clonal lymphatic malignancies preceded by a polyclonal lymphoproliferative stage, which is usually reversible by reducing immunosuppression. The distinction between these stages is sometimes difficult to assess. Immunologists have so far failed to find a lymphatic tumor-specific antigen, hence, monoclonality is usually based on a constellation of factors, namely homogeneity of the phenotypic expression of few antigens, aberrant expression of antigens and restricted expression of kappa- or lambda-chains in malignancies expressing surface immunoglobulins. Nonrandom chromosomal translocations as well as other aberrations, usually important in the diagnosis of malignancy, are sometimes of limited value. This is mainly due to the existence of translocations [like t(14;18) and t(2;5)] in nonmalignant states, and their non-specificity [the existence of t(8;14) in Burkitt's lymphoma and large cell lymphoma, t(2;5) in Hodgkin's disease and anaplastic large cell lymphoma, and t(14;18) in large cell lymphoma evolving from follicular lymphoma and Burkitt's lymphoma]. The diagnostic tools available in 1995, although usually sufficient, are sometimes unable to distinguish between malignancy and reactivity. Some problematic cases will be more accurately defined as lying in the gray zone, or as belonging to a spectrum ranging between reactivity and malignancy.
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PMID:The gray zone between malignant and reactive processes in lymphoproliferative diseases. 887 7

Lymphoid neoplasia is a complex area comprising multiple diseases with varied pathology, treatment, and outcome. The non-Hodgkin's lymphomas are reviewed here. Non-Hodgkin's lymphomas, collectively, represent the sixth most common cancer in the United States as well as the sixth most common cause of cancer deaths. The overall incidence of non-Hodgkin's lymphoma has risen steadily over the past four decades. Although some of this is attributable to human immunodeficiency virus (HIV)-associated lymphoma, HIV-associated disease accounts for only a small part of the increase in lymphoma. As our knowledge of normal as well as neoplastic lymphoid development has expanded on the basis of histopathology as well as adjunct cellular and molecular techniques, multiple classifications have been proposed to take these into account. The clinical relevance to our understanding of non-Hodgkin's lymphoma is the concept that various lymphoid cancers are counterparts of stages of normal lymphoid development. Stages of lymphoid development in terms of cell surface markers and immunoglobulin gene rearrangements have been well characterized. These are particularly applicable to the early B-cell development, which is antigen-independent and occurs in the bone marrow. Diseases correlating with these stages are largely acute lymphocytic and lymphoblastic leukemia/lymphoma and high-grade lymphomas, such as Burkitt's lymphomas. Much has been learned recently about subsequent antigen-dependent B-cell development in secondary lymphoid organs to improve our understanding of the corresponding stages of B-cell neoplasia. Many of these stages correlate with more recently described entities such as mantle cell and marginal zone lymphomas. Histologic study remains crucial in determining the subtype of NHLs, whereas immunohistochemistry, surface phenotype, and molecular studies are useful in selected cases. Although some lymphoma classifications may be better in terms of understanding the lymphoma biology, the working formulation remains useful to guide clinical decision making. Lymphomas classified as low grade are considered incurable with standard therapy when diagnosed, as is usual, at advanced stages. Different subtypes may have different median survivals, but the goal has typically been palliation, whereas experimental approaches are clearly needed. Intermediate and high-grade lymphomas are potentially curable with aggressive combination chemotherapy. Recent evidence suggests that CHOP chemotherapy is as effective as more complex regimens. Still, 40% to 50% of patients are cured. Prognostic factor analysis has allowed separation of subgroups with much better survival in whom CHOP is adequate versus those with much poorer survival in whom experimental approaches are rational. Additional subtypes of lymphomas have been described and characterized since the working formulation was developed, including mucosa-associated lymphoid tissue tumors (MALT-oma), mantle zone lymphoma, anaplastic large cell lymphoma and AILD-like T-cell lymphoma. Approaches to these entities are still being optimized. Newer approaches, including high-dose therapy with stem cell support, biologic agents, and newer chemotherapeutic agents are discussed, as are special situations such as localized lymphoma of certain sites and lymphoma in immunosuppressed patients.
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PMID:Non-Hodgkin's lymphoma. 891 70

The Epstein-Barr virus, a human herpesvirus, has been found in the neoplastic cells of numerous lymphoid malignancies, including Burkitt's lymphoma, immunodeficiency-associated lymphoproliferative disorders, nasal T/NK lymphoma, and Hodgkin's disease. The available data suggest that Epstein-Barr virus contributes to the pathogenesis of many of these neoplasms but is not directly linked to the etiology of any of these lymphomas.
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PMID:The association of the Epstein-Barr virus with malignant lymphoma. 899 Nov 18

In addition to endemic Burkitt's lymphoma and nasopharyngeal carcinoma a variety of other epithelial-and lymphoid-derived proliferative diseases has been shown to closely link with Epstein-Barr virus (EBV) infection. The former include thymic lymphoepithelial carcinoma, oral hairy leukoplakia and gastric carcinoma. The latter include B-cell lymphoproliferative disorders arising in individuals with primary or secondary immunodeficiency, Hodgkin's disease and T/NK lymphoma. The significance of serological, immunohistochemical and molecular biological methods such as PCR, in situ hybridization and Southern blotting is described. And also the possible prognostic impact of EBV in T/NK-lymphoma is discussed.
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PMID:[Current diagnosis and prognostic importance of EBV-associated neoplasms]. 904 39


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