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Query: UMLS:C0079731 (B-cell lymphoma)
 16,671 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We studied the clinicopathologic features and treatment outcome of patients with breast diffuse large B-cell lymphoma. As to the cellular immunophenotype, CD5 was detected in two patients, CD10 in 4, BCL2 in 20, BCL6 in 11, and MUM-1 in 17. The 5-year progression-free survival was 77% and the 5-year overall survival was 87%. Patients with the germinal center B-cell (GCB) type had a significantly better prognosis than those with the non-GCB type. The combination of anthracycline-containing chemotherapy and/or involved-field radiotherapy produced a relatively good prognosis. However, it is a heterogeneous disease with regard to histological type and pathological state.
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PMID:Clinicopathologic features and treatment outcome of primary breast diffuse large B-cell lymphoma. 1849 56

Follicular lymphoma (FL) is one of the most common subtypes of non-Hodgkin lymphoma and frequently transforms to diffuse large B-cell lymphoma (DLBCL). To clarify some aspects of the natural history of FL, we retrospectively examined 43 consecutive patients who had DLBCL with pre- or coexisting FL grade 1 or 2. The patients comprised 22 men and 21 women with a median age of 53 years. Most of the patients (34/43) showed advanced-stage (III or IV) disease initially. We examined both FL and DLBCL components morphologically, immunohistochemically, and by interface fluorescence in situ hybridization (FISH: IGH/BCL2 fusion, BCL6 translocation) analysis. Most of the DLBCLs were classified as the centroblastic subtype, with two exceptions of the anaplastic subtype. Immunohistochemical analysis of both the FL and DLBCL components revealed the following respective positivity rates: CD20 100%/100%, CD10 86%/66%, Bcl-2 96%/91%, Bcl-6 84%/88%, MUM1 16%/34%, CD30 0%/20%, CD138 0%/0%, and CD5 0%/3%. Loss of CD10 (6/36, 17%) and gain of MUM1 (7/28, 25%) and CD30 (5/21, 24%) through transformation were not infrequent. High positivity rates for Bcl-2 and Bcl-6 were maintained throughout transformation. Among the DLBCLs, 84% were classified as the germinal center B-cell phenotype (GCB) and 16% as non-GCB in accordance with the criteria of Hans et al. IGH/BCL2 fusion was detected by FISH in 89% of FLs and 82% of DLBCLs. BCL6 translocation was detected in 1/6 (17%) DLBCLs without IGH/BCL2 fusion. Thus, although the morphological features and FISH results for DLBCL were consistent with transformed FL, the immunophenotype showed wide heterogeneity.
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PMID:Diffuse large B-cell lymphoma after transformation from low-grade follicular lymphoma: morphological, immunohistochemical, and FISH analyses. 1854 5

The clinicopathologic features of malignant lymphoma of the small intestine were reviewed. Genetically, characteristic chromosomal translocations have been identified in several B-cell lymphomas, such as t (11 ; 18)/API2-MALT1 in MALT lymphoma, t (14 ; 18)/IGH-BCL2 in follicular lymphoma, or t (3 ; 14)/BCL6-IGH in diffuse large B-cell lymphoma (DLBCL). Histologically, DLBCL is most frequently observed, and T-cell lymphoma and follicular lymphoma are more frequent in small intestinal cases than in gastric cases. Macroscopically, small intestinal lymphomas are classified as polypoid, ulcerative (including stricturing, non-stricturing and aneurysmal forms on radiography), multiple lymphomatous polyposis, diffuse, or other types. A significant correlation is observed between these macroscopic/radiographic and histologic types. The therapeutic strategy, such as surgery, chemotherapy, antibiotics or watch-and-wait, should be determined based on the disease extent, histologic type, and clinical stage.
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PMID:[Malignant lymphoma of the small intestine]. 1861 20

Lymphoplasmacytic lymphoma (LPL) is a small B-cell lymphoma with plasmacytic differentiation that does not fulfill the criteria for any other small B-cell lymphoma. Cytogenetic characterization of nodal LPL is limited and the distinction from marginal zone lymphomas with plasmacytic differentiation can be problematic. Thus, 17 cases of lymph node-based LPL were studied with fluorescence immunophenotypic and interphase cytogenetics for the investigation of neoplasia (FICTION) using a CD79a antibody and probes to detect trisomies of chromosomes 3 (15 cases), 12 (16 cases), and 18 (17 cases); rearrangements (R) of IgH (10 cases), BCL6 (6 cases), PAX5 (7 cases), and MALT1 (16 cases); and deletion 6q21 (7 cases). Cases with IgH R were further studied with an IgH/BCL2 probe. In cases without FICTION studies, previously reported fluorescence in situ hybridization results for IgH, PAX5, and deletion 6q21 were available from prior studies. The histopathology, immunophenotype, and available clinical data were also reviewed. Three pathologic categories were recognized: 5 classic LPL, 5 vaguely nodular polymorphous (VN-P), and 7 other. Among the classic LPL, 4/4 had an IgM paraproteinemia, 5/5 had bone marrow involvement (BM+), and 1/5 had +MALT1. One of one VN-P LPL had an IgM paraprotein, 2/4 were IgM+, 2/4 IgG+, 1/3 had BM+, and 1/5 had an IgH R. Among the other cases, 2/3 had a paraprotein, 2/7 were IgM+, 5/7 IgG+, and 0/3 had BM+. Of these cases, 1 showed +12, 1 +18, and 1 IgH/BCL2 rearrangement plus +18. None of the 17 cases had a 6q21 deletion or +3. Therefore, with rare exception, lymph node-based LPL with classic or more varied histopathologic features does not have the cytogenetic abnormalities frequently associated with bone marrow-based LPL/Waldenstrom macroglobulinemia or many of the marginal zone lymphomas. The search for better objective inclusionary criteria for LPL must continue.
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PMID:Fluorescence immunophenotypic and interphase cytogenetic characterization of nodal lymphoplasmacytic lymphoma. 1867 Mar 52

Diagnosis and classification of aggressive mature B-cell lymphoma with atypical morphology remains a challenge. To identify factors that may contribute to the atypical morphology, we selected eight such cases and evaluated their morphologic, immunophenotypic and cytogenetic features and clinical outcomes. The neoplastic cells showed a diffuse monotonous infiltrating pattern with a spectrum of morphology including: 1) L1 lymphoblastic; 2) centroblastic; 3) immunoblastic; and 4) mixed centroblastic and immunoblastic. The lymphoma cells in most cases were positive for CD10 and/or BCL6, and showed BCL2 expression. 6 of 8 cases showed C-MYC rearrangements, and interestingly, all 6 cases demonstrated a proliferation index of < or =90%. 3 of the 6 cases also demonstrated t(14;18). Clinical follow-up indicated that aggressive mature B-cell lymphoma may benefit from more intensified chemotherapeutic regimens used for BL. Our study suggests that aggressive mature B-cell lymphoma with atypical morphology may be another "grey zone lymphoma" lying in the spectrum between Burkitt lymphoma and diffuse large B-cell lymphoma.
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PMID:C-MYC rearrangements are frequent in aggressive mature B-Cell lymphoma with atypical morphology. 1878 24

We present a case of diffuse large B-cell lymphoma CD5 negative, Cyclin D1 positive presenting as ruptured spleen in a 63-year-old man requiring emergent splenectomy. Tumor cells showed marked pleomorphism, anaplasia, and increased mitotic figures with positive Cyclin D1, BCL6, MUM1, P53, and a high MIB1 proliferative fraction. The patient received multiple therapies and ultimately died. This case raises the differential diagnoses of pleomorphic mantle cell lymphoma and other aggressive lymphomas with pleomorphic, anaplastic, and Reed-Sternberg-like cells.
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PMID:CD5 negative, Cyclin D1-positive diffuse large B-cell lymphoma (DLBCL) presenting as ruptured spleen. 1883 19

Cytogenetic abnormalities of chromosome 12p involving the TEL/ETV6 gene are observed in a variety of hematopoietic neoplasms including acute leukemias, myelodysplastic syndromes, and myeloproliferative disorders. Karyotypic aberrations, including rearrangements, deletions, and amplifications of chromosome 12p, have been documented in B-cell non-Hodgkin lymphoma; however, rearrangements targeting TEL have rarely been reported. Here we describe a diffuse large B-cell lymphoma that had a complex karyotype including t(9;12)(q22;p13), which was confirmed by fluorescence in situ hybridization to represent rearrangement of TEL. Additional cytogenetic abnormalities included t(3;14)(q27;q32) involving the variant, alternative breakpoint region of the BCL6 gene and del(6)(q13q23), resulting in the loss of 1 allele of BLIMP1. This case reiterates the importance of correlating morphologic and phenotypic findings with the results of cytogenetic analysis to avoid errors in diagnosing hematologic neoplasms and highlights the rare association of B-cell non-Hodgkin lymphoma with aberrations of TEL.
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PMID:Diffuse large B-cell lymphoma with TEL/ETV6 translocation. 1899 13

BCL6 is a transcriptional repressor that is overexpressed in diffuse large B-cell lymphoma and follicular lymphoma. The N-terminal POZ domain of BCL6 interacts with transcriptional corepressors and targeting these associations is a promising therapeutic strategy. Previous structural studies of the BCL6 POZ domain have used a mutant form because of the low solubility of the wild-type recombinant protein. A method for the purification and crystallization of the wild-type BCL6 POZ domain is described and the crystal structure to 2.1 A resolution is reported. This will be relevant for the design of therapeutics that target BCL6 POZ-domain interaction interfaces.
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PMID:Structure of the wild-type human BCL6 POZ domain. 1905 59

Epstein-Barr virus positive diffuse large B-cell lymphoma (EBV+ DLBCL) of the elderly is a rare B-cell lymphoproliferative disorder (B-LPD) that occurs in patients > 50 years with no known history of immunodeficiency or lymphoma. Patients present with moderate to severe clinical B-symptoms. These lesions show complete effacement of normal tissue/nodal architecture by large atypical lymphoid cells/immunoblasts and Hodgkin/Reed-Sternberg-like giant cells with variable amounts of inflammatory cells in the background. The ratio of neoplastic to inflammatory cells, degree of mitoses and necrosis can be quite variable; hence EBV+ DLBCL of the elderly was historically divided into low grade polymorphic and high grade monomorphic types. Further studies have shown both types to be different points in the spectrum of disease, and are all high grade lymphomas. The neoplastic large lymphoid cells show expression of CD20/CD79a and PAX-5, with variable expression of CD30, LMP-1 and EBNA-2, but CD15, CD10 and BCL6 are generally negative. Neoplastic cells show EBER positivity and high Ki-67 expression. Differential diagnoses include EBV+ B-LPD, classical Hodgkin lymphoma and EBV-DLBCL. EBV+ DLBCL of the elderly is highly aggressive with a median survival of 2 years. These patients are less responsive to standard chemotherapy compared with other B-LPD.
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PMID:Epstein-Barr virus positive diffuse large B-cell lymphoma of the elderly. 1925 22

Chromosomal translocations are the hallmark genetic aberration in non-Hodgkin lymphoma (NHL), with specific translocations often selectively associated with specific NHL subtypes. Because many NHL-associated translocations involve cell cycle, apoptosis, and lymphocyte development regulatory genes, we evaluated NHL risk associated with common genetic variation in 20 candidate genes in these pathways. Genotyping of 203 tag single nucleotide polymorphisms (SNP) was conducted in 1,946 NHL cases and 1,808 controls pooled from 3 independent population-based case-control studies. We used logistic regression to compute odds ratios (OR) and 95% confidence intervals (CI) for NHL and four major NHL subtypes in relation to tag SNP genotypes and haplotypes. We observed the most striking associations for tag SNPs in the proapoptotic gene BCL2L11 (BIM) and BCL7A, which is involved in a rare NHL-associated translocation. Variants in BCL2L11 were strongly related to follicular lymphoma only, particularly rs3789068 (OR(AG), 1.41; 95% CI, 1.10-1.81; OR(GG), 1.65; 95% CI, 1.25-2.19; P(trend) = 0.0004). Variants in BCL7A were strongly related to diffuse large B-cell lymphoma only, particularly rs1880030 (OR(AG), 1.34; 95% CI, 1.08-1.68; OR(AA), 1.60; 95% CI, 1.22-2.08; P(trend) = 0.0004). The associations for both variants were similar in all three studies and supported by haplotype analyses. We also observed notable associations for variants in BCL6, CCND1, and MYC. Our results support the role of common genetic variation in cell cycle, apoptosis, and lymphocyte development regulatory genes in lymphomagenesis, and suggest that effects may vary by NHL subtype. Replication of our findings and further study to identify functional SNPs are warranted.
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PMID:Risk of non-Hodgkin lymphoma associated with germline variation in genes that regulate the cell cycle, apoptosis, and lymphocyte development. 1933 52


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