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 defects in lymphocyte apoptosis that underlie the autoimmune lymphoproliferative syndrome (ALPS) are usually attributable to inherited mutations of the CD95 (Fas) gene. In this report, we present the histopathological and immunophenotypic features seen in the lymph nodes (n = 16), peripheral blood (n = 10), bone marrow (n = 2), spleen (n = 3), and liver (n = 2) from 10 patients with ALPS. Lymph nodes showed marked paracortical hyperplasia. Interfollicular areas were expanded and populated by T cell receptor-alphabeta CD3+ CD4-CD8- (double-negative, DN) T cells that were negative for CD45RO. CD45RA+ T cells were increased in all cases studied. The paracortical infiltrate was a result of both reduced apoptosis and increased proliferation, as measured by in situ detection of DNA fragmentation and staining with MIB-1, respectively. The paracortical proliferation may be extensive enough to suggest a diagnosis of malignant lymphoma. Many of the paracortical lymphocytes expressed markers associated with cytotoxicity, such as perforin, TIA-1, and CD57. CD25 was negative. In addition, most lymph nodes exhibited florid follicular hyperplasia, often with focal progressive transformation of germinal centers; in some cases, follicular involution was seen. A polyclonal plasmacytosis also was present. The spleens were markedly enlarged, more than 10 times normal size. There was expansion of both white pulp and red pulp, with increased DN T cells. DN T cells also were observed in liver biopsies exhibiting portal triaditis. In the peripheral blood, the T cells showed increased expression of HLA-DR and CD57 but not CD25. CD45RA+ T cells were increased in the four cases studied. Polyclonal B cell lymphocytosis with expansion of CD5+ B cells was a characteristic finding. Taken together, the histopathological and immunophenotypic findings, particularly in lymph nodes and peripheral blood, are sufficiently distinctive to suggest a diagnosis of ALPS. Of note, two affected family members of one proband developed lymphoma (T-cell-rich B-cell lymphoma and nodular lymphocyte predominance Hodgkin's disease, respectively).
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PMID:Pathological findings in human autoimmune lymphoproliferative syndrome. 981 46

The morphologic spectrum of large B-cell lymphoma is broad. Several unusual variants have been described such as lymphoma with myxoid stroma, sclerosing B-cell lymphoma, signet ring-cell lymphoma, and multilobated B-cell lymphoma among others. We report on five cases of cutaneous large B-cell lymphoma in which the neoplastic cells were spindle-shaped. In two cases, the clinical features fulfilled those of a primary cutaneous lymphoma; in the three other cases, the lymphoma most likely arose primarily in the skin, but incomplete clinical workups precluded definite categorization. The patients ranged in age from 30 to 89 years and presented with solitary lesions on the trunk or head. Histopathologic examination revealed nodular or dense diffuse infiltrates involving the entire dermis as well as the subcutaneous fat in some cases. Thickened collagen bundles between the spindled cells were present in one case. Cytomorphologic analysis showed the presence of round or oval medium-sized and large-sized lymphocytes with features of centrocytes and centroblasts in some foci, with others dominated by cells with spindle-shaped, elongated, twisted nuclei with dispersed chromatin and scant cytoplasm. Immunohistologic analysis revealed that both round or oval and spindled cells were positive for CD20 in all cases; in all cases tested, these cells were also positive for MIB-1 and were negative for CD3, CD5, CD43, CD45RO, CD21, CD30, CD68, S-100, HMB-45, actin, smooth-muscle actin, and cytokeratin. Bcl-2 was expressed in one of three cases tested. Analysis of the rearrangement of the J(H) gene by polymerase chain reaction performed in one case showed a monoclonal pattern. Spindle-cell large B-cell lymphoma represents a distinctive rare subtype of the cutaneous large B-cell lymphoma and can arise primarily in the skin in some cases. Recognition of this variant is necessary to avoid misdiagnosis of other cutaneous malignant spindle-cell tumors.
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PMID:Cutaneous spindle-cell B-cell lymphoma: a morphologic variant of cutaneous large B-cell lymphoma. 1245 11

The bcl-2 oncogene plays an important role in carcinogenesis by inhibiting cell death (apoptosis). It was initially discovered in follicular B cell lymphoma with t(14,18) and subsequently found in other malignant and premalignant lesions. Alteration of the normal controls of cell proliferation is also a significant factor in the multistep process of tumorigenesis. The proliferative activity of a given lesion is commonly evaluated by MIB 1, a monoclonal antibody to Ki67 proliferation antigen. Mutation of the p53 gene is considered the most common genetic aberration in colorectal cancer. Immunohistochemical (IHC) staining expression of bcl-2, Ki67, and p53 was retrospectively investigated in a series of 52 colorectal carcinomas and 56 adenomas. The aim of the study was twofold: (i) to investigate any correlation between MIB 1, p53, and bcl-2 immunostaining expression in colonic adenomas and carcinomas and (ii) to identify any relation between these markers and several histopathologic parameters including tumor size, pathologic stage, lymph node metastasis, angiolymphatic invasion, tumor grade, and differentiation in colon carcinomas. bcl-2 was consistently higher in adenomas than in carcinomas. There were 44 of 56 (78.6%) adenomas and 27 of 52 (51.9%) carcinomas positive for bcl-2 (P = 0.004). The mean Ki67 labeling index (LI) was 30.05 +/- 7.6 and 38.12 +/- 11.01 in adenomas and carcinomas, respectively (P = 0.0001). p53 was significantly higher in carcinomas (35 of 52 [67.3%]) than in adenomas (18 of 56 [32.1%]) (P = 0.0004). Expression of bcl-2 in carcinoma was associated with a lower p53 levels and lower mean Ki67 LI and with favorable histopathologic parameters. Higher p53 and Ki67 values were associated with prognostically poor histopathologic features (differentiation and Duke's stage). We conclude that, in contrast to p53 and Ki67, bcl-2 oncoprotein expression is probably an early step in the process of colon carcinogenesis, and its expression may be associated with favorable pathologic parameters. Furthermore, an inverse relation exists between p53 and Ki67, and bcl-2 IHC expression in colonic neoplasia. Evaluation of bcl-2, p53, and Ki67 IHC expression in colonic carcinoma may be of value in predicting the clinical course in these patients.
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PMID:Immunohistochemical expression of bcl-2 and p53 oncoproteins: correlation with Ki67 proliferation index and prognostic histopathologic parameters in colorectal neoplasia. 1098 68

We investigated chromosomal alterations using comparative genomic hybridization (CGH), and DNA ploidy patterns using laser scanning cytometry (LSC) in 8 primary central nervous system lymphomas (PCNSLs). The average number of chromosomal alterations detected by CGH was 6.9 (gain: 4.1, deletion: 2.8). Frequent alterations were gains of chromosomes 12, 18q, and X, and deletion of 6q, which were similar to those seen in non-CNS diffuse large B-cell lymphoma. DNA aneuploidy was detected by LSC in 4 of the 8 cases. The DNA aneuploid lymphomas had more chromosomal alterations than the DNA diploid ones (9.3 vs. 4.5, P <.05). The former had higher MIB-1 indices than the latter. The present investigation indicates that although most of the PCNSL are histologically uniform, they are divided cytogenetically into DNA aneuploid and diploid tumors.
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PMID:Distinct primary central nervous system lymphoma defined by comparative genomic hybridization and laser scanning cytometry. 1136 58

We hereby present a retrospective clinicopathological and immunohistochemical study of surgically resected primary gastrointestinal (GI) lymphoma with an analysis of parameters of potential prognostic relevance. From a larger series of 144 cases of primary GI lymphomas, we chose 61 cases with sufficient clinical follow-up (mean 60, range 1-219 months), classified either as extranodal marginal zone B-cell lymphoma of MALT type (MALT lymphoma) or diffuse large B-cell lymphoma (DLBCL), after having excluded other subtypes. In addition to conventional clinical and morphological parameters, the expression levels of Ki-67 (MIB-1), bcl-2 and p53 were evaluated for prognostic significance. Twenty-one (34.4%) cases were classified as pure low grade marginal zone B-cell lymphoma of MALT type, 12 (19.7%) cases as low grade MALT lymphoma with a high grade component (mixed type), and 28 (45.9%) cases as primary extranodal DLBCL. Most of the lymphomas (53/61; 86.9%) were localized in the stomach, 3 (4.9%) in the small bowel, 3 (4.9%) multifocal in both stomach and small intestine and 2 (3.3%) in the large bowel. MIB-1 expression in more than 30% of tumor cells was detected in 42 (68.6%), bcl-2 expression in 20 (32.8%) and p53 accumulation in more than 10% of neoplastic cells in 16 (26.2%) lymphomas. Both high Ki-67 expression and p53 accumulation were more prevalent in the DLBCL. 30 (49%) patients showed lymph node involvement at surgery, 14 (23%) patients suffered tumor recurrence, and 24 (38.5%) died during the follow-up period. Tumor recurrence occurred primarily in patients who had presented lymph node involvement (9/14, 64.3%). The 5-year survival rate was 66.1% for all patients. Important prognostic factors for overall survival were tumor stage (p < .004) and p53 accumulation (p < .05) in univariate analysis, and tumor stage in multivariate analysis (p < .001). Although p53 accumulation did not reach statistical significance in our small study group, it may be both important in the transformation of low grade MALT lymphoma and an indicator for aggressive behavior in high grade tumors.
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PMID:Primary gastrointestinal B-cell lymphoma. A clincopathological and immunohistochemical study of 61 cases with an evaluation of prognostic parameters. 1143 65

Gastrobiopsy specimens obtained from 15 patients, in whom gastroscopy examination pointed to gastric cancer and primary gastric lymphoma was later diagnosed in surgical material after gastrectomy, were immunostained using the anti MIB-1 antibody to assess the proliferative index. A control study was carried out on representative surgical specimens from these patients and on 10 gastrobiopsy specimens from patients in whom histopathology revealed moderate/severe gastritis. The proliferative activity was assessed with respect to the type of lymphoma (primary gastric marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue MALT type vs. diffuse large B-cell lymphoma) and stage of the lymphoma, and we compared the proliferative indices between inflammatory infiltrate and lymphoma cells. There were significant differences in the proliferative indices between inflammatory infiltrate and lymphoma cells, with immunohistochemical reactivity for MIB-1 antigen being visible even in the case of markedly mechanically damaged (crushed) gastrobiopsy material and accompanying necrosis. There were also differences in the proliferative indices between the groups of lymphomas varying in stage, although without statistical significance. There were no statistical differences in the rate of proliferation between lymphomas varying in "malignancy". The present results argue for the auxiliary role of the assessment of proliferative activity in gastrobiopsy material facilitating differentiation between inflammatory infiltrate and neoplastic infiltrate, especially in cases where mechanical damage or necrosis do not allow for precise histopathological evaluation.
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PMID:Evaluation of the proliferative activity as an adjuvant helpful tool in the diagnosis of primary gastric lymphoma in gastrobiopsy. 1191 82

To compare immunophenotypic and molecular features between Burkitt lymphoma (BL) and diffuse large B-cell lymphoma (DLBCL) with c-myc rearrangements (c-mycR DLBCL), we analyzed 18 cases of B-cell non-Hodgkin's lymphoma with c-mycR that were confirmed by chromosomal and/or Southern blotting analyses. The cases were histologically classified into 10 BLs and five DLBCLs. The remaining three cases could not be classified because of suboptimal quality of the surgical materials. BLs were from five adults and five children, whereas all DLBCLs were from adults. BLs were positive for CD20 (10/10 cases examined), CD10 (9/10), Bcl-2 (1/9), and Bcl-6 (10/10), whereas they were negative for CD3 (0/10) and EBV (0/8), by Epstein-Barr virus (EBV) EBER-1 RNA in situ hybridization. c-MycR DLBCLs were positive for CD20 (5/5), CD10 (2/5), Bcl-2 (3/4), and Bcl-6 (4/4), whereas none of them were positive for CD3 and EBV. A mean of MIB-1 index (MIB-1+ cells/neoplastic cells, %) of BLs (98.1%) was higher than that of c-mycR DLBCLs (66.3%; P <.0001). Somatic mutation of immunoglobulin heavy-chain gene variable region (VH gene) in BLs (four cases) ranged from 0.7 to 4.9% with an average value of 2.3%, whereas those in DLBCLs (three cases) from 8.2 to 32.0% with an average value of 17.0%. It is, therefore, concluded that a growth fraction of nearly 100%, as well as a monotonous proliferation of medium-sized cells and c-myc(R), should be of value in the diagnosis of BL, which is probably different from c-myc(R) DLBCL. In addition, CD10+, Bcl-2-, and low frequency of mutation of the VH gene could be helpful for the histologic distinction of BL from (c-mycR) DLBCL.
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PMID:The distinction between Burkitt lymphoma and diffuse large B-Cell lymphoma with c-myc rearrangement. 1211 16

We examined 13 cases of the primary central nervous system malignant lymphoma (PCNSML) with established histological diagnosis and valid clinical information to clarify the clinicopathologic characteristics of PCNSML. All tumors were classified according to the World Health Organization as diffuse large B-cell lymphoma (DLBCL), and four were positive for CD 10 or BCL-6. All of the tumors had a high proliferative activity containing 15.7% to 84.8% (mean = 59.8%) MIB-1 positive cells, although there was no correlation between MIB-1 staining indices and the survival period of patients. Molecular analysis using PCR demonstrated clonal rearrangement of the IgH gene in the majority of cases analyzed (9/10 = 90%). Tumor cell apoptosis was consistently demonstrated by the routinely-processed histology and the immunohistochemistry for single-stranded DNA, and apoptotic cells were engulfed by phagocytic macrophages. These findings indicate that the majority of this series of PCNSML were DLBCL with high proliferative activity and clonal B-cell process and that a subset of the cases are of putative germinal center B-cell origin. Since there was no statistical correlation between the histopathological finding and the survival rate, additional studies, including quantitative investigation for the extent of apoptotic cells, in large numbers of patients are required.
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PMID:[Clinicopathologic characteristics of primary central nervous system malignant lymphoma]. 1247 78

Human T-lymphotropic virus type I (HTLV-I) is closely associated with T-cell lymphoma/leukaemia, which always shows monoclonal HTLV-1 provirus DNA integration. HTLV-1 is not associated with B-cell lymphoma. The relationship between B-cell lymphoma and HTLV-1 was analysed retrospectively in early stage B-cell non-Hodgkin's lymphoma (NHL) according to HTLV-1 infection and pathological features. We analysed 198 cases of head and neck B-cell NHL treated with radiotherapy and/or chemotherapy; 21 were seropositive and 177 were seronegative for HTLV-1. We also immunostained 26 cases of diffuse large B-cell lymphoma (DLBL), including 12 seropositive and 14 seronegative for HTLV-1 respectively, for CD20, CD3, CD4, CD8, CD56, MIB-1 and T-cell-restricted intracellular antigen (TIA-1) to examine the phenotype, immunity and proliferation activity. The 5-year overall survival rates were 78% and 49% (P = 0.007, log rank test) for HTLV-1 seronegative and seropositive cases respectively. Infection with HTLV-1 was significantly associated with poor survival in patients with B-cell lymphoma by multivariate analysis. For DLBL, HTLV-1 infection was not a significant factor, but the overall survival curve was similar to that of the 21 seropositive B-cell lymphoma cases. Lymphoma cells were negative for TIA-1, but the background lymphocytes were positive for this marker. The number of TIA-1-positive cells was higher in HTLV-1-negative cases than in-positive cases. In conclusion, patients with B-cell-NHL (B-NHL) who are also HTLV-1 carriers have a poorer prognosis than non-carriers. HTLV-1 does not seem to be associated with lymphomagenesis of the B phenotype itself, but correlates with host immunity by reducing the number of cytotoxic T-cells.
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PMID:HTLV-1 carriers with B-cell lymphoma of localized stage head and neck: prognosis, clinical and immunopathological features. 1461 63

Cdc25B and cdc25A phosphatases are representative stimulators of cell cycle progression, and recent studies have also indicated their oncogenic roles. In this study, we investigated the expression of these phosphatases in malignant lymphoma of the thyroid by immunohistochemistry. These phosphatases were not expressed in follicular cells in normal follicles, but were heterogeneously or diffusely expressed in the follicles in chronic thyroiditis and malignant lymphoma. In infiltrating lymphocytes in chronic thyroiditis, they were only occasionally expressed. Of the 47 cases of lymphoma, 30 (63.8%) were classified as high group for cdc25B because it was expressed in more than 25% of lymphoma cells. Cdc25B expression level was inversely associated with MIB-1 labeling index (p=0.0008), and aberrant p53 expression (p=0.0077). Furthermore, cases of marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MZBL) were more frequently classified as high group (p=0.0318) than those of diffuse large B-cell lymphoma (DLBL). On the other hand, 22 cases (46.8%) were regarded as high group for cdc25A, but its expression level was not linked to those parameters. These findings suggest that i) cdc25B plays a role in the early phase of thyroid lymphoma possibly including the malignant transformation from chronic thyroiditis, and ii) cdc25A may contribute to the progression of lymphoma.
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PMID:Cdc25A and cdc25B expression in malignant lymphoma of the thyroid: correlation with histological subtypes and cell proliferation. 1476 75


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