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Query: UNIPROT:Q06643 (non-Hodgkin's lymphoma)
 11,307 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Anaplastic large cell lymphoma (ALCL) is a subtype of non-Hodgkin's lymphoma characterized by the CD30+ large neoplastic cells and sometimes carries a t(2;5)(p23;q35). Recently, we found a novel hyperphosphorylated 80-kD protein tyrosine kinase, p80, in ALCLs with t(2;5). Subsequent cDNA cloning showed p80 to be a fusion protein of two genes, the novel tyrosine kinase gene and the nucleophosmin gene, in accordance with the sequence of the NPM/ALK gene (Morris et al, Science 263:1281, 1994). Meanwhile, the clinicopathologic features of p80-carrying ALCLs have remained unclear. Paraffin sections of 105 cases of ALCL were immunostained using anti-p80 antibody, and 30 of them were shown to express p80. Clinicopathologic comparison between p80-positive and -negative ALCLs showed that p80-positive cases occurred in a far younger patient age group (16.2 +/- 12.9 years; p80-negative cases, 51.0 +/- 22.3 years; P < .0001) and the patients showed a far better 5-year survival rate (79.8%; p80-negative group, 32.9%; P < .01). These data showed that p80-positive ALCL is a distinct entity both clinically and pathogenetically and should be differentiated from p80-negative ALCL.
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PMID:Anaplastic large cell lymphomas expressing the novel chimeric protein p80NPM/ALK: a distinct clinicopathologic entity. 765 22

The cytoplasmic C-terminus of Epstein-Barr virus (EBV) latent infection membrane protein 1 (LMP1) is essential for B lymphocyte growth transformation and is now shown to interact with a novel human protein (LMP1-associated protein 1 [LAP1]). LAP1 is homologous to a murine protein, tumor necrosis factor receptor-associated factor 2 (TRAF2), implicated in growth signaling from the p80 TNFR. A second novel protein (EBI6), induced by EBV infection, is the human homolog of a second murine TNFR-associated protein (TRAF1). LMP1 expression causes LAP1 and EBI6 to localize to LMP1 clusters in lymphoblast plasma membranes, and LMP1 coimmunoprecipitates with these proteins. LAP1 binds to the p80 TNFR, CD40, and the lymphotoxin-beta receptor, while EBI6 associates with the p80 TNFR. The interaction of LMP1 with these TNFR family-associated proteins is further evidence for their role in signaling and links LMP1-mediated transformation to signal transduction from the TNFR family.
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PMID:The Epstein-Barr virus transforming protein LMP1 engages signaling proteins for the tumor necrosis factor receptor family. 785 81

The classification of malignant lymphoma has been based on morphological and immunophenotypical findings for a long time. Recently, as chromosomal and genomic abnormalities which closely relate to the specific subtypes of lymphoma are revealed, these factors becoming much more important in the evaluation of differences in clinicopathological features of the various lymphoma subtypes. Anaplastic large cell lymphoma (ALCL) is a subtype of non-Hodgkin's lymphoma (NHL) involving large CD30+ neoplastic cells, which occasionally carries the chromosomal translocation t(2;5)(p23;q35). We have recently found a novel hyperphosphorylated 80-kDa protein tyrosine kinase, p80 which is expressed specifically in human ALCLs with this translocation. Subsequent cDNA cloning showed p80 to be a fusion protein of two genes, the novel tyrosine kinase gene and the nucleophosmin gene, in accordance with the sequence of the NPM/ALK gene. In order to clarify the clinicopathologic features of p80-carrying ALCLs, we developed an anti-p80 polyclonal antibody, which immunoprecipitated, immunoblotted and immunostained p80 specifically. When paraffin sections of 105 cases of ALCL were stained using the anti-p80 antibody, 30 were shown to be p80 positive Clinicopathological comparison between p80-positive and p80-negative ALCLs revealed that the p80-positive cases occurred in a much younger patient age group and that the patients showed a far better 5-year survival rate. These data suggest that p80-positive ALCL is a distinct entity and should be differentiated from p80-negative ALCL.
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PMID:The clinicopathological features of anaplastic large cell lymphomas expressing p80NPM/ALK. 902 82

CD30(+)-anaplastic large cell lymphoma (ALCL) is a distinct clinicopathologic entity of non-Hodgkin's lymphoma that is immunologically heterogeneous. Bimodal age distribution, a nonrandom chromosome abnormality [t(2;5)(p23;q35)] that produces a chimeric protein p80npm/alk, and a variable (5-47%) association with Epstein-Barr virus (EBV) have been reported. We reviewed 36 cases of ALCL (19 were children < 20 yr and 17 were adults) by focusing on the presence or absence of p80npm/alk protein and EBV. Immunophenotyping studies were performed on frozen and/or paraffin sections before initiation of chemotherapy. The p80 protein was immunohistochemically examined, and EBV-encoded RNA transcripts were detected by in situ hybridization. Among 19 cases in children, 13 cases had a T-cell and 6 cases had a null-cell phenotype. p80 Was detected in 16 (84.2%) of 19 cases in children and 3 (17.6%) of 17 cases in adults. All of the 19 cases of p80-positive ALCL were positive for epithelial membrane antigen, regardless of age. EBV genome was not detected in any of 19 cases in children and in only 2 of 15 cases in adults. ALCL in childhood seems to constitute a homogeneous entity characterized by expression of p80 and absence of EBV. The association of EBV is also infrequent in adult ALCL among Japanese patients.
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PMID:CD30-positive anaplastic large cell lymphoma in childhood: expression of p80npm/alk and absence of Epstein-Barr virus. 907 28

Cytogenetic investigations were performed in a case of a nodal malignant non-Hodgkin's lymphoma. Histopathological analysis from an involved lymph node as well as from a skin biopsy revealed a lymphohistiocytic variant of CD30-positive anaplastic large cell lymphoma (ALCL). A t(2;5)(p23;q35) chromosome translocation could be observed in all metaphases analysed. This finding was confirmed both by RT-PCR analysis of the NPM/ALK fusion protein and by positive staining with the p80(NPM/ALK) antibody. To the best of our knowledge, this is the first report of a t(2;5) documented by classic cytogenetics in the lymphohistiocytic variant of ALCL.
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PMID:A lymphohistiocytic variant of anaplastic large cell lymphoma with demonstration of the t(2;5)(p23;q35) chromosome translocation. 945 Aug 9

A recurrent, reciprocal balanced translocation, t(2;5) (p23;q35), has been recognized in CD30+ anaplastic large-cell lymphomas (ALCL), a newly recognized subtype comprising approximately 5% of all non-Hodgkin's lymphoma (NHL). This translocation creates a novel fusion protein, NPM-ALK, which has transforming properties in vitro and can cause large-cell lymphoma in vivo when transfected into murine bone marrow. Multiple techniques including reverse transcriptase-polymerase chain reaction (RT-PCR) amplification of NPM-ALK fusion transcripts, genomic DNA-PCR, RNA in situ hybridization, and fluorescence in situ hybridization (FISH) of metaphase chromosomes and interphase nuclei, and immunohistochemical detection of the 80 kilodalton protein (p80) derived from the NPM-ALK fusion have enabled surveys of normal and lymphoma tissues for evidence of the translocation. These studies suggest that expression of ALK protein, a novel orphan receptor tyrosine kinase, is normally confined to the nervous system. In lymphoma, NPM-ALK expression is most often seen in young patients with the monomorphic or small-cell variant of ALCL who present with advanced stage disease and have tumors with a CD30+, T- or null-cell phenotype. It is less frequently detected in older patients and in ALCL of pleomorphic histology. In addition, expression of NPM-ALK has been found in occasional CD30 negative B-cell lymphomas with diffuse large cell or immunoblastic histology. NPM-ALK is rarely, if ever, detected in Hodgkin's disease or secondary ALCL. Although initially found in primary nodal ALCL, recent studies suggest that NPM-ALK expression may occur in lymphoma at extranodal sites, including the skin; it remains controversial, however, whether CD30+ primary cutaneous lymphoma and its benign counterpart, lymphomatoid papulosis (LyP), express NPM-ALK in some cases. A retrospective study has suggested that expression of NPM-ALK is associated with a better overall 5-year survival; these results must be confirmed in prospective studies of patients with uniform staging and therapy to more fully understand the clinical significance of the t(2;5) and its novel chimeric protein, NPM-ALK.
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PMID:The t(2;5) in human lymphomas. 968 23

The clinicopathological and biological significance of Hodgkin's disease and non-Hodgkin's lymphoma, which are infrequently encountered in women of childbearing age, remains to be clarified. We recently reviewed 4 cases of non-Hodgkin's lymphoma of the T/natural killer (T/NK)-cell phenotype, all of which were associated with pregnancy and characterized by the expression of the cytotoxic granule-associated proteins T-cell intracellular antigen-1 and/or granzyme B. The 4 cases selected had presented between November 1993 and May 1999. The criteria for selection were that the onset of clinical manifestations occurred during pregnancy or within 6 months after delivery. The patients comprised 1 patient with p80/anaplastic lymphoma kinase (ALK)-positive anaplastic large cell lymphoma (ALCL), 1 with p80/ALK-negative ALCL, and 2 with peripheral T/NK-cell lymphomas of unspecified type. The diseases followed aggressive clinical courses: 3 patients died within 6.5 months after diagnosis, and only 1 was still alive with the disease 17 months after diagnosis. The diseases appeared to progress rapidly after delivery. Maternal immunity and hormonal changes during pregnancy may be closely related to the biological behavior of these unusual tumors. This study is, to the best of our knowledge, the first to address pregnancy-associated cytotoxic lymphoma.
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PMID:Pregnancy-associated cytotoxic lymphoma: a report of 4 cases. 1159 20