Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:3.4.24.11 (CD10)
 9,792 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Nine patients with t(11;14) and B non-Hodgkin's lymphomas composed of small to intermediately sized cells with irregular nuclei are described. Immunophenotyping was performed on seven cases, which were M+, D- with light chain restriction, CD5+, CD10-, and CD20+, suggesting that they were non-follicle centre cell lymphomas. The translocation (11;14) (in three cases the only cytogenetic anomaly) was associated with rearrangement of bcl-1 in four of the five cases investigated. Translocation (11;14) has been described in an apparently heterogeneous group of low-grade lymphoid malignancies which we suggest have a non-follicle centre cell lineage in common. This translocation may be associated with these lymphomas in the same way that t(14;18) is associated with follicle centre cell lymphomas.
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PMID:Translocation (11;14): a cytogenetic anomaly associated with B-cell lymphomas of non-follicle centre cell lineage. 200 19

Mantle cell lymphomas (MCLs) are typically CD5-expressing B-cell non-Hodgkin's lymphomas (NHLs) that frequently harbor the chromosomal translocation t(11;14) or bcl-1 gene rearrangements. Insufficient data are available on the biologic features and clinical behavior of rigorously characterized MCL. As these NHLs have been reported to exhibit various histologic and cytologic expressions, and in order to avoid using somewhat arbitrary and subjective morphologic definitions, we chose to study cases of MCL selected on more objective grounds. Specifically, 15 samples (from 14 patients) of CD5-expressing B-cell NHLs with detectable bcl-1 gene rearrangement were included. Overall, these patients had relatively uniform clinical manifestations. Most were older men (mean age, 67 years) who presented with lymphadenopathy, high-stage disease, and bone marrow involvement. All but two patients relapsed, demonstrated residual tumor, or had disease progression after an initial response to various therapies. Nine patients have died; these patients had a median survival of only 19 months. All cases could be classified within the broad morphologic spectrum previously described for MCL, and no predominant histologic subtype was observed. However, cases could be segregated into two major groups according to tissue architecture: one with a purely diffuse pattern and the other with at least a focal nodular component. Patients with purely diffuse tumors had a lower survival rate (0%) than those with tumors having a nodular component (62% survival rate). In contrast to the morphologic variability, these NHL exhibited a rather homogeneous immunophenotypic pattern. All cases demonstrated intense CD20 expression, with typically intense IgM and light chain expression, and relatively weak IgD expression. In no case was CD10 detected on the neoplastic cells. DNA content analysis showed aneuploidy only in three instances, and two groups of cases could be arbitrarily defined on the basis of their S-phase fraction. A relationship between a purely diffuse growth pattern and a high S-phase fraction (greater than 5%) was observed. As expected from this association, patients with tumors having high S-phase fractions fared worse (14% survival rate) than those patients with tumors showing lower S-phase fractions (57% survival rate). Thirteen NHLs from 12 patients had amplifiable bcl-1 gene rearrangements at the major translocation cluster (MTC). The bcl-1 breakpoints aggregated within a 63-bp region of the MTC, and the amplified tumor DNA from each patient had unique N-nucleotide junctional sequences and Ig joining region breakpoint sites.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:CD5-expressing B-cell non-Hodgkin's lymphomas with bcl-1 gene rearrangement have a relatively homogeneous immunophenotype and are associated with an overall poor prognosis. 753 38

Diffuse large B cell lymphomas (DLBLs) represent a heterogeneous collection of aggressive non-Hodgkin's lymphomas that can arise either de novo or as a result of transformation from chronic lymphocytic leukemia, small lymphocytic lymphoma, follicular lymphomas, or lymphomas of mucosa-associated lymphoid tissue. A small percentage of DLBLs express the CD5 antigen. The majority of these cases have evolved from a pre-existing low grade non-Hodgkin's lymphoma (Richter's syndrome). However, we identified and characterized nine CD5-positive DLBLs in which the patients did not have a previous history or concomitant evidence of chronic lymphocytic leukemia, small lymphocytic lymphoma, follicular lymphoma, or mucosa-associated lymphoid tissue-associated non-Hodgkin's lymphoma, suggesting that they arose de novo. All nine cases expressed CD20 and monotypic immunoglobulin, all eight cases examined expressed CD19, CD22 and CD43, eight of the nine cases expressed HLA-DR, and two of eight cases expressed CD11c. None of the cases expressed CD3, CD10, CD11b, CD21, CD23 or CD30. CD5 expression by these cells was found to be identical to that of CD5-positive B cell chronic lymphocytic leukemia by quantitative polymerase chain reaction analysis of CD5 mRNA. These nine de novo CD5-positive DLBLs exhibited clonal immunoglobulin heavy and light chain gene rearrangements but lacked integration of the Epstein-Barr virus genome and structural alterations of the bcl-1, bcl-2, c-myc, H-ras, K-ras, and N-ras proto-oncogenes and the p53 tumor suppressor gene. However, bcl-6 proto-oncogene rearrangement, which is involved in chromosome band 3q27 aberrations, was found in four cases (44.4%). This is comparable with the frequency of bcl-6 gene rearrangement in CD5-negative DLBL. In contrast, bcl-6 gene rearrangement was absent in six cases of DLBL associated with Richter's syndrome. These findings suggest that de novo CD5-positive DLBLs are genotypically similar to CD5-negative DLBLs and may be pathogenetically distinct from the DLBLs associated with Richter's syndrome.
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PMID:De novo CD5-positive and Richter's syndrome-associated diffuse large B cell lymphomas are genotypically distinct. 754 11

We have investigated the cellular origin and/or pathogenesis of follicular small cleaved cell lymphoma (FSCCL), diffuse small cleaved cell lymphoma (DSCCL) and intermediate lymphocytic lymphoma/lymphocytic lymphoma of intermediate differentiation (ILL/IDL) based on a series of immunologic and molecular genetic (bcl-1, bcl-2 and bcl-3 genes) studies. These studies have led to the conclusion that the cellular origin or pathogenesis of ILL/IDL and DSCCL is distinctly different from that of FSCCL: (1) FSCCL is a neoplastic counterpart of follicular center cells (FCC) of secondary follicles because of the presence of CD10 and bcl-2 gene rearrangement and the absence of CD5 and bcl-1 gene rearrangement; (2) DSCCL and ILL/IDL are a neoplastic counterpart of mantle zone (MZ) B lymphocytes because of the presence of CD5 and bcl-1 gene rearrangement and absence of CD10 and bcl-2 gene rearrangement; and (3) FSCCL scarcely develops into DSCCL, and the previously proposed concept that DSCCL represents a diffuse counterpart of FSCCL does not hold good. These results indicate that DSCCL and ILL/IDL are identical, derived from primary follicular cells or MZB cells of secondary follicles, and should be unified under MZB lymphocyte-derived lymphomas. They are distinguished from FCC-derived lymphomas in morphologic, immunologic, cytogenetic and molecular genetic features. Bcl-1 and bcl-2 genes may be associated with the pathogenesis of FCC-derived lymphoma and MZB lymphocyte-derived lymphoma, respectively.
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PMID:Histogenesis and pathogenesis of follicular small cleaved cell lymphoma (FSCCL), diffuse small cleaved cell lymphoma (DSCCL) and intermediate lymphocytic lymphoma/lymphocytic lymphoma of intermediate differentiation (ILL/IDL). 764 69

The morphology, phenotype, genotype and clinical behaviour of four cases of mantle cell lymphoma (centrocytic lymphoma) presenting primarily in mucosa (two gastric, one in large bowel and one tonsillar) are reviewed. Their relationship with the broader group of mantle cell and mucosa-associated lymphoid tissue (MALT) lymphomas is also discussed. All four tumours showed a monomorphic picture of mantle cells (centrocytes) arranged in a diffuse, or vaguely nodular, pattern. Scattered non-neoplastic germinal centres were entrapped within the tumour cells, although there was no follicular colonization. In two cases distinct epithelial infiltration by tumour cells was observed. All four tumours had a CD19, CD20, CD5, IgD, Leu8 immunophenotype, whereas KiM1P and CD10 expression were absent. DRC antibody showed loose aggregates of dendritic cells in three of four cases. Three cases showed PRAD-1/Cyclin D1 overexpression by Northern blot analysis. Although we were not able to detect bcl-1 rearrangement in the major translocation cluster (MTC) breakpoint, the possibility of bcl-1 rearrangement involving other cluster breakpoints cannot be ruled out. The four cases evolved as a disseminated disease, involving either peripheral lymph nodes, spleen or bone marrow. The biological behaviour of mantle cell lymphoma presenting in mucosa appears, irrespective of localization or macroscopic presentation, similar to that of nodal mantle cell lymphoma. Their tendency to dissemination contrasts with MALT lymphomas, which tend to remain localized, and from which mucosa mantle cell lymphoma must be distinguished. The presence of lymphoepithelial lesions does not seem to be a useful differential feature, since occasional epithelial infiltration was seen in two cases. Reactivity with CD5 appears to be especially useful in distinguishing these, since all four cases were clearly positive, in contrast with what is usually found in MALT lymphomas.
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PMID:Mucosal mantle cell (centrocytic) lymphomas. 765 10

Ten cases of classic centrocytic lymphoma as defined in the Kiel classification system were investigated for their immunophenotype, their proliferation activity and by means of molecular diagnostics. The findings were compared to those obtained from a group of nine cases of anaplastic centrocytic lymphoma. Both groups showed virtually identical immunohistochemical characteristics with positivity for CD5 and negativity for CD10 and CD23. In the group of anaplastic centrocytic lymphoma, there were considerably higher proliferation indices as documented by staining for the Ki-67 antigen, up to 80% of the tumour cells being positive. Moreover, the cases of anaplastic centrocytic lymphoma had bcl-1 gene rearrangements in eight out of nine cases compared with three out of 10 cases of classic centrocytic lymphoma. DNA analysis was not able to detect bcl-2 gene rearrangement in any case, pointing to a difference compared with lymphomas of germinal centre origin. The coincidence of anaplastic and sometimes blast-like morphology of the tumour cells, high proliferation index and a rearranged bcl-1 gene in nearly all cases of anaplastic centrocytic lymphoma support their classification as high-grade malignant variants of centrocytic lymphoma and suggest a possible role for the bcl-1 locus not only in the origin but also in the progression of centrocytic lymphomas.
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PMID:The anaplastic variant of centrocytic lymphoma is marked by frequent rearrangements of the bcl-1 gene and high proliferation indices. 804 22

Flow immunophenotyping, DNA content analysis, and polymerase chain reaction (PCR) amplification for t(11;14) and t(14;18) were performed on 11 cases of typical mantle cell lymphoma (MCL), 5 cases of apparent MCL with proliferation centers (MCL-PC), and 5 cases of small lymphocytic lymphoma (SLL). Immunophenotyping showed IgM (P < .001), Ig light (P < .001), and CD20 (P < .001) expression to be more intense in MCL than in SLL. In MCL-PC, the mean intensity of IgM, Ig light chain, and CD20 expression was intermediate to the intensities observed in MCL and SLL. Furthermore, in contrast to SLL, all MCL and 4 of 5 MCL-PC cases exhibited stronger CD20 than CD19 expression. CD10 expression was not observed in any case and CD5 expression was present in all SLL and MCL-PC cases and in 9 of 11 MCL cases. DNA content analysis showed an S-phase fraction of less than 3% in all cases studied and, except for 1 MCL case, all lymphomas were DNA diploid. The t(11;14) breakpoint junctions involving the bcl-1 major translocation cluster were amplified by PCR in 4 of 11 (36%) MCL cases and in none of the MCL-PC or SLL cases. The t(14;18) involving the bcl-2 major breakpoint region was not identified by PCR in any case. We conclude that the level of expression of surface antigens and the rapid detection of t(11;14) by PCR are potentially useful for distinguishing MCL and SLL in the clinical setting. Further investigations as to the biologic relationship between MCL, MCL-PC, and SLL, and the utility of t(11;14) PCR in these lymphomas are warranted.
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PMID:Antigen expression and polymerase chain reaction amplification of mantle cell lymphomas. 812 54

Histological subtyping of non-Hodgkin's lymphomas is of prognostic significance. Current classification systems subdivide them into low-, intermediate-, and high-grade malignancies. These subgroups are largely supported by clinical findings, but immunophenotyping and genotyping have shown that several of these histologically defined subcategories are heterogeneous. This is exemplified by the 'diffuse small cleaved-cell lymphoma' which comprises B-cell and T-cell lymphomas. Moreover, within the B-cell lymphomas, several entities have been included, which recapitulate the different compartments present in the reactive B follicle, e.g., the follicle centre, the mantle, and the marginal zone. Mantle-cell lymphomas have been identified in the United States as mantle-zone lymphomas and as intermediate differentiated lymphomas and in Europe as centrocytic lymphomas. Morphology, immunophenotyping, and cytogenetics of these three lymphomas support their similarity and underline their distinction from follicle centre-cell lymphomas as well as from small lymphocytic lymphomas. All three are composed of a mixture of small round and small cleaved cells, expressing several B-cell markers, surface immunoglobulins, and CD5, but lacking CD10 expression. They often carry the translocation t(11;14) with rearrangement of bcl-1/PRAD1 gene. The behaviour and responsiveness to therapy of mantle-cell lymphomas has not been fully documented yet. In order to obtain these data, precise subtyping of non-Hodgkin's lymphomas--not only based on morphology, but supported by immunophenotyping and cytogenetic analysis--is now mandatory.
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PMID:Mantle-cell lymphoma. 817 14

Large-cell variants are uncommon in mantle cell lymphoma (MCL). Here we describe the pathologic and clinical findings in five patients with large-cell lymphoma related to MCL (L-MCL), and compare them to a group of classic small-cell MCL (s-MCL) cases. Histologically, the MC origin of the large cells was evinced by their association with a small mantle cell component in the same tissue, or their distribution in a classic mantle zone pattern, or their development in a patient with previous s-MCL. The large cells were either pleomorphic mantle cells (case 1) or transformed blast-like cells (case 2-5). The median nuclear diameter, median nuclear area and proliferation index of L-MCLs and s-MCLs, were statistically different. Immunophenotypic characterization of four specimens of L-MCL and 10 of s-MCLs with a large panel of antibodies showed the classic findings of MCL, i.e. the IgM+ D+/-, CD5+, CD10-, CD23- phenotype in all cases except two (one CD5- and one CD23+), and the association with a loose follicular dendritic cell network. Two of four L-MCLs and 5/10 s-MCLs demonstrated rearrangements of the bcl-1 gene by Southern blot or by polymerase chain reaction (PCR); 2/4 L-MCLs and 1/9 s-MCLs had p53 mutations on single-strand conformation polymorphism analysis; none of the 14 specimens showed rearrangement of bcl-2 by PCR or bcl-6 and c-myc by Southern blot. All patients with 'transformed' histology (versus 37% of all others) died of lymphoma; their survival (15-18 months; median 17) was much shorter than that of all the others (28-117+ months; median 43) (P=0.0035). All three patients with p53 anomalies, two of whom had tumours with transformed histology, died of their disease in a short time (15, 18 and 28 months). In contrast, the presence of bcl-1 rearrangements did not have prognostic implications. This study documents the existence of large-cell variants of MCL and the poor prognosis associated with the 'transformed' cytologic type and/or p53 abnormalities.
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PMID:Large-cell variants of mantle cell lymphoma: cytologic characteristics and p53 anomalies may predict poor outcome. 863 52

We examined a 63-year-old female, who was referred to our hospital for a thorough examination of abnormal findings on the X-ray which were first detected in May, 1992, and suggested a tumor. After hospital admission, a bracheobronchial lymph node biopsy was performed, and the results suggested a pseudo-lymphoma. However, because the possibility of a malignant tumor was not completely negated and the patient, as well as her family, hoped to receive surgical treatment, we performed the tumor resection. The tumor had a clear border. S6 was 3 x 3.5 x 4 cm, S8 was 2 x 2.5 x 3 cm, and evaluation according to the tumor node metastasis (TNM) classification was T1 N0 M0, i.e., Stage I. In addition to an ordinary histopathological examination, an immunohistochemical examination was conducted for CD19+, CD20+, CD22+, IgD-, CD5-, CD10-. Overall, the tumor was diagnosed as a MALT lymphoma. Investigated IgH, TCR beta, bcl-1 and bcl-2 genes did not show reconstitution. Because it is difficult to differentiate a malignant lymphoma, primarily developed in the lung, from a MALT lymphoma on a morphological examination of a tissue specimen, immunohistochemical and genetic examinations are helpful for making a diagnosis.
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PMID:[A case of mucosa associated lymphoid tissue (MALT) lymphoma]. 893 47


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