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)

The high expression of the T-cell oncogene TCL1 in B-cell tumors and the emergence of B-cell lymphomas in TCL1-transgenic mice suggest a pathogenetic role for this kinase coregulator in B-cell malignancies. We compared the expression of TCL1 in B-cell tumors with their differentiation stage. As with normal B-cell subsets, uniform TCL1 expression was characteristic of tumors of pregerminal center derivation such as precursor B-cell lymphoblastic leukemia/lymphoma (85%, 47/55) and mantle cell lymphoma (84%, 49/58), and was more variable in follicular lymphoma (57%, 28/49). Large B-cell lymphoma was less frequently positive for TCL1 (36%, 18/50), especially among cases of the activated B-cell type. All types of Hodgkin lymphoma, splenic marginal zone lymphoma, and post-germinal center-derived tumors, including plasma cell myeloma and MALT lymphoma, were negative for TCL1, except for 1 case. In nearly all TCL1-expressing tumors, as with normal B cells, variations in cellular TCL1 levels were related to the proliferation and microenvironmental factors. In normal B cells, cell lines and primary B-cell tumor samples, TCL1 downmodulation occurred after prolonged cytokine treatment and/or B-cell receptor stimulation. In contrast to mature T-cell tumors where TCL1 expression is always indicative of an activating TCL1 gene translocation, TCL1 expression in B-cell tumors parallels its regulation in non-neoplastic B cells. Therefore, TCL1 expression can be used diagnostically as an indicator of the differentiation stage of a given B-cell tumor.
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PMID:TCL1 in B-cell tumors retains its normal b-cell pattern of regulation and is a marker of differentiation stage. 1759 80

Herein, we report on a patient with splenic marginal zone lymphoma who initially presented without splenomegaly and bone marrow (BM) or peripheral blood involvement. At first, the patient showed moderate leukothrombocytopenia; she was completely asymptomatic, and BM examination excluded a hematologic disease. After 7 months, spleen enlargement was detected without determining any symptoms or worsening of the bilinear cytopenia. Bone marrow histologic, immunohistochemic, cytologic, and immunophenotypic examinations were normal. Splenectomy was performed, and a diagnosis of splenic marginal zone B-cell lymphoma was established. A monoclonal IgVH gene rearrangement was identified in the spleen tissue (VH3 gene family) and subsequently detected in the BM mononuclear cells. Because of the large surgical debulking and the absence of histologic, cytologic, and immunophenotypic BM involvement, no further treatment was proposed. After the splenectomy, the blood cell count normalized, and neither BM nor peripheral blood involvement appeared after 12 months of follow-up.
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PMID:An insidious presentation of splenic marginal zone lymphoma. 1762 11

Splenic marginal zone lymphoma with or without villous lymphocytes (SLVL/SMZL) is a low grade B-cell lymphoma that affects patients in the sixth decade and has a median survival greater than 10 years. A substantial proportion of patients die from causes unrelated to the disease. Close to a third of the patients do not require intervention and a policy of watch and see is reasonable and recommended. There are several therapeutic options that have proved effective in these patients. Due to the natural history of the lymphoma, the main goal of all these treatments is to achieve control of the disease rather than its eradication. Retrospective designs of all documented studies, the lack of uniform response criteria and the heterogeneity in the patient's features makes interpretation of the data difficult. Splenectomy remains one of the first line options in patients fit for surgery. Amongst chemotherapy, purine analogues, in particular fludarabine in combination or not with Rituximab and Rituximab alone have a greater efficacy than alkylating agents in terms of achieving better quality of response and longer progression free survival; therefore these agents are recommended particularly in patients who are not candidates for surgery or relapse after splenectomy. In the small proportion of patients with concomitant hepatitis C virus (HCV) infection, Interferon-alpha, ribavirin or a combination of both has demonstrated a significant activity with responses correlating with clearance of HCV RNA in the blood; therefore, these agents should be considered in the therapeutic scenario as a first line in these small cohort of patients. Patients that transform to high-grade lymphoma and the minority that have TP53 abnormalities should be treated with other schedules. Prospective randomized trials would be desirable to ascertain the independent prognostic factors and the biological features that predict disease progression and drug resistance to device the optimal management and treatment for SLVL/SMZL.
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PMID:Splenic marginal zone lymphoma with and without villous lymphocytes. 1768 Feb 18

GCET1 (germinal center B cell-expressed transcript-1) gene codes for a serpin expressed in germinal center (GC) B cells. Following the observation that follicular lymphoma cases exhibit an increased level of Gcet1 expression, compared with follicular hyperplasia, we have characterized Gcet1 protein expression in human tissues, cell lines, and a large series of lymphomas. To this end, we have performed immunohistochemical and Western blot analyses using a newly generated monoclonal antibody that is reactive in paraffin-embedded tissues. Our results demonstrate that Gcet1 is expressed exclusively by neoplasms hypothetically to be arrested at the GC stage of differentiation, including follicular lymphoma, nodular lymphocyte predominant Hodgkin lymphoma, and a subset of diffuse large B-cell lymphoma, T-cell/histiocyte rich B-cell lymphoma, and Burkitt lymphoma. Within these tumors, Gcet-1 protein expression is restricted to a subset of GC B cells, establishing the existence of a distinct heterogeneity among normal and neoplastic GC B cells. None of the other B-cell lymphomas, that is, chronic lymphocytic leukemia, splenic marginal zone lymphoma, and mantle cell lymphoma, was Gcet1(+), which underlines the potential utility of Gcet1 expression in lymphoma diagnosis. The results of RNA and protein expression should prompt further investigation into the role of Gcet1 in regulating B-cell survival.
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PMID:Gcet1 (centerin), a highly restricted marker for a subset of germinal center-derived lymphomas. 1789 15

We compared the morphologic findings of different types of marginal zone B-cell lymphoma (MZL) involving the bone marrow (BM), including 18 splenic (SMZL), 6 extranodal (mucosa-associated lymphoid tissue lymphoma), and 6 nodal cases. The median percentage of BM involvement was 15%, and multiple overlapping patterns of infiltration were observed in all MZL types. The most frequent patterns were nodular (87%) and interstitial (63%). A focal sinusoidal pattern of involvement was found in one third of SMZLs and rarely in MALT lymphoma. Germinal centers (GCs) were uncommon in routinely stained BM biopsy sections and were observed only in SMZL. However, antibodies specific for CD21 and CD23 highlighted follicular dendritic cells in most MZLs of all types. MZLs cannot be distinguished from each other by examining BM sections alone. However, a sinusoidal pattern or presence of GCs is suggestive of SMZL. Furthermore, correlation with the CBC count can further enhance the reliability of diagnosing SMZL.
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PMID:Bone marrow involvement by marginal zone B-cell lymphomas of different types. 1842 30

In a series of 48 patients with splenic marginal zone lymphoma (SMZL) with circulating villous lymphocytes, we describe the clinical and laboratory features of nine cases that transformed to high-grade B-cell lymphoma. These patients had a significantly greater incidence of peripheral lymph node involvement at diagnosis when compared to SMZL patients who did not transform (P < 0.03). While transformation in the bone marrow is frequently refractory to therapy and associated with poor outcome in SMZL, lymph node transformation responds well to chemotherapy with durable progression-free and overall survival.
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PMID:High-grade transformation in splenic marginal zone lymphoma with circulating villous lymphocytes: the site of transformation influences response to therapy and prognosis. 1867 6

The assembly of a collection of gene-expression signatures of the major types of B-cell non-Hodgkin's lymphoma has identified increased T-cell leukemia/lymphoma 1A (TCL1) expression in multiple lymphoma types and cases, and has enabled the investigation of the functional and clinical importance of TCL1 expression. Specifically, Burkitt's lymphoma cases show a homogeneously strong expression of TCL1, whereas diffuse large B-cell lymphoma, follicular lymphoma, mantle cell lymphoma, chronic lymphocytic leukemia, nodal marginal zone lymphoma, and splenic marginal zone lymphoma display a striking variability in the intensity of TCL1 staining. This was validated in two independent series. A Gene-Set Enrichment Analysis of the genes correlated with TCL1A expression found that variation in the level of expression of TCL1A was significantly associated with some of the most important gene signatures recognizing B-cell lymphoma pathogenesis and heterogeneity, such as germinal center, B-cell receptor, NF-kappaB (and its target genes), death, MAP kinases, TNFR1, TOLL, and IL1R. Additionally, TCL1 expression was correlated with shorter time to treatment in chronic lymphocytic leukemia cases and shorter lymphoma-specific survival in mantle cell lymphoma series, thus indicating the clinical and biological significance of TCL1 expression, and suggesting TCL1A as a potential therapeutic target.
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PMID:TCL1A expression delineates biological and clinical variability in B-cell lymphoma. 1882 Jun 75

Immunoglobulin gene usage and somatic mutation patterns were studied in 59 patients with splenic marginal zone lymphoma and were correlated with clinical characteristics. Fifty-nine IGHV rearrangements were amplified. IGHV1, IGHV3, and IGHV4 subgroups accounted for 30%, 56%, and 14% of sequences, respectively. IGHV genes most frequently used were IGHV1-2 (n=12), IGHV3-23 (n=15), IGHV3-30 (n=7) and IGHV4-34 (n=5). IGHV was unmutated in 25%. Villous lymphocytes >10% were detected in 50% of patients belonging to the IGHV1-2 group, in 21% of the IGHV3-23 group, and in no patient of the IGHV3-30 group (p=0.05). Liver involvement was present in 50% of the IGHV3-30 group, in 9% of the IGHV3-23 group, and in no patient of the IGHV1-2 group (p=0.04). HCV-serology was positive in 50% of the IGHV3-30 group, in 7% of the IGHV3-23 group, and in 17% of the IGHV1-2 group (p=0.04). The proportion of intermediate and high risk patients according to the SMZL score was higher in the unmutated respect to the mutated group (69% vs 32%, p=0.05). In conclusion, IGHV rearrangement analysis in splenic marginal zone B-cell lymphoma reveals a non-random preference for use of IGHV1-2, IGHV3-23 and IGHV3-30 genes, whose presence differs according to clinical features and prognostic category.
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PMID:Splenic marginal zone lymphoma: Clinical clustering of immunoglobulin heavy chain repertoires. 1925 Aug 48

Lymphomas are classified according to the World Health Organisation (WHO) classification which defines subtypes on the basis of clinical, morphological, immunophenotypic, molecular and cytogenetic criteria. Differential diagnosis of the subtypes is sometimes difficult, especially for small B-cell lymphoma (SBCL). Standardisation of molecular genetic assays using multiple gene expression analysis by microarrays could be a useful complement to the current diagnosis. The aim of the present study was to develop a low density DNA microarray for the analysis of 107 genes associated with B-cell non-Hodgkin lymphoma and to evaluate its performance in the diagnosis of SBCL. A predictive tool based on Fisher discriminant analysis using a training set of 40 patients including four different subtypes (follicular lymphoma n = 15, mantle cell lymphoma n = 7, B-cell chronic lymphocytic leukemia n = 6 and splenic marginal zone lymphoma n = 12) was designed. A short additional preliminary analysis to gauge the accuracy of this signature was then performed on an external set of nine patients. Using this model, eight of nine of those samples were classified successfully. This pilot study demonstrates that such a microarray tool may be a promising diagnostic approach for small B-cell non-Hodgkin lymphoma.
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PMID:Evaluation of a low density DNA microarray for small B-cell non-Hodgkin lymphoma differential diagnosis. 1929 57

Splenic marginal zone B-cell lymphoma is characterized by high genetic heterogeneity, and hepatitis C virus infection seems to be involved in a subset of patients. The aims of the analysis were to identify potential genetic alterations related to hepatitis C virus status, IgV(H) gene mutational status, and prognostic categories identified in a multicenter study (Blood 2006;107:4643). Genome-wide array comparative genomic hybridization at a 100-kilobase (kb) resolution was performed in 34 patients with splenic marginal zone B-cell lymphoma, 12 of whom were hepatitis C virus positive. Array-comparative genomic hybridization experiments revealed no copy number alterations in 10 patients (4 were hepatitis C virus positive). A median of 5.6 and 3.8 copy number alterations were detected in hepatitis C virus-positive and in hepatitis C virus-negative patients, respectively. The most frequent copy number alterations involved chromosomes 7 and 17 (21% and 24%, respectively). Except for Xp gain (P = .01), no differences in common alterations were found between hepatitis C virus-positive and hepatitis C virus-negative cases. Unmutated status of the IgV(H) gene was related to del(7q) (P = .04) and dup(12q) (P = .03). The high-risk group identified according to the new splenic marginal zone B-cell lymphoma prognostic score was associated with del(7q) (P = .01) and del(17p) (P = .02). Hepatitis C virus-positive splenic marginal zone B-cell lymphoma patients have no specific chromosome alterations. Patients with poor prognosis are characterized by distinctive imbalances.
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PMID:High-resolution genome-wide array comparative genomic hybridization in splenic marginal zone B-cell lymphoma. 1964 53


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