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)

Primary cutaneous marginal zone B-cell lymphoma (PCMZL) is included as one of the major types of primary cutaneous B-cell lymphoma in the revised World Health Organization-European Organization for Research and Treatment of Cancer classification. Clinically, PCMZL is an indolent disease and has an excellent prognosis. PCMZL is composed of a polymorphous infiltrate that includes centrocyte-like, monocytoid, and lymphoplasmacytoid lymphocytes and plasma cells. Numerous reactive T cells and lymphoid follicles are commonly associated with the neoplasm. The neoplastic cells express B-cell markers and usually bcl-2 and are negative for CD5, CD10, and bcl-6. Borrelia burgdorferi is a suspected etiologic agent identified in a subset of cases. Although all of these neoplasms presumably are monoclonal, monoclonal IgH rearrangement can only be detected in approximately 75% of cases. Most molecular studies to assess for clonality have used polymerase chain reaction-based methods, and thus this false-negative rate may be attributable to somatic mutation of the IgH variable region genes. Approximately 10% to 20% of PCMZLs have recurrent chromosomal translocations, including the t(14;18)(q32;q21)/IgH-malt1, t(11;18)(q21;q21), and t(3;14)(p14;q32). The t(14;18)(q32;q21) and t(11;18)(q21;q21) have been shown to activate the NF-kappaB pathway.
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PMID:Primary cutaneous marginal zone B-cell lymphoma. 1683 Sep 56

Mantle cell lymphoma (MCL) is a rare B-cell lymphoma that has never been characterized in Taiwan. The purpose of the present paper was to retrospectively identify 21 cases in male patients, with a median age of 61, involving lymph node (91%), marrow (71%), and peripheral blood (23%). Eighteen (86%) were in stages III/IV with 1 and 5 year survival rates of 78% and 17%, respectively. Mixed nodular and diffuse pattern (45%) was most common while interstitial pattern (92%) predominated in marrow. Eighteen (86%) were of classical morphology, two were pleomorphic and one was blastic. The tumors expressed IgM and bcl-2 (100%), cyclin D1 (95%), CD5 (86%), CD43 and IgD (62%), CD52 (60%), and bcl-6 (5%). Ki-67 index>or=30% (P=0.1834) was associated with a trend toward poorer survival while p21, p27, or p53 expression was not statistically significant for survival. Real-time polymerase chain reaction for cyclin D1 (CCND1) gene mRNA expression showed high levels in nine cyclin D1-positive patients and a low level in the single cyclin D1-negative patient. The latter patient was cyclin D2 positive and negative for immunoglubuin heavy chain gene and CCND1 gene translocation by locus-specific interphase fluorescent in situ hybridization. In conclusion, it is confirmed that the usual morphological variants and aberrant immunophenotype of MCL in the West occur in Taiwan and that this disease carries a poor prognosis.
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PMID:Mantle cell lymphoma in Taiwan: clinicopathological and molecular study of 21 cases including one cyclin D1-negative tumor expressing cyclin D2. 1687 38

A member of the family of p53-related genes, p63 plays a role in regulating epithelial proliferation and differentiation programs, but the pathological and clinical meaning of p63 in B-cell lymphoma has not been elucidated. We investigated the expression pattern of p63 in B-cell malignancies, and evaluated the correlation between the expression of p63 and other germinal center markers. Ninety-eight B-cell lymphomas (28 FCL, 5 MCL, and 65 DLBCL) were analyzed by immunohistochemical examination for p63, bcl-6, CD10 and MUM-1 proteins, and for rearrangement of bcl-2/IgH. Expression of p63 was observed in the nuclei of tumor cells obtained from 15 of 28 (54%) FCL, 22 of 65 (34%) DLBCL, but none of 5 MCL. In DLBCL, the expression of p63 and bcl-6 showed a significant correlation (P < 0.02), but no correlation was observed between p63 and expression of CD10, MUM-1, or bcl-2/IgH rearrangement. RT-PCR revealed that TAp63alpha-type transcripts, a possible negative regulator of transcriptional activation of p21 promoter, were major transcripts in B-cell lymphoma tissues. As for prognostic significance, only patients in the p63 positive group of FCL died, and in the non-germinal center group, the p63 positive cases appeared to have inferior overall survival than other groups in DLBCL. Our preliminary results suggested that p63 expression is a disadvantageous factor for prognosis in this subgroup of B-cell lymphomas.
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PMID:Clinico-pathological characteristics of p63 expression in B-cell lymphoma. 1691 93

Advances in molecular biology have provided an increased understanding of the heterogeneity of diffuse large B-cell lymphoma, allowing multiple clinical and biologic prognostic factors to be elucidated. Recently, the addition of rituximab to CHOP (cyclophosphamide/doxorubicin/vincristine/prednisone) has been adopted as the new standard of care, representing the first major improvement in therapy in 2 decades. Although outcomes have markedly improved, patients with lymphoma that is not cured with this standard first-line therapy continue to pose a difficult challenge. Early identification of patients at high risk would allow alternative treatment strategies to be considered in a risk-based management approach. Accurate risk assessment will require all previously recognized prognostic indicators to be revalidated in the era of immunochemotherapy. Although the International Prognostic Index remains predictive, it no longer distinguishes a subgroup with < 50% chance of survival. Many molecular prognostic markers, such as Bcl-2 and Bcl-6 protein expression, no longer appear predictive of outcome. Early positron emission tomography scanning is a powerful independent predictive tool that will likely be relied on more frequently in the future. Finally, the role of increased dose intensity or dose density will need to be reevaluated for combinations that include rituximab. Alternative treatment strategies and newer therapies will need to be explored in the context of clinical trials.
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PMID:Treatment of diffuse large B-cell lymphoma: a risk-based approach. 1710 Oct 68

Chromosomal translocations affecting band 3q27, where BCL6 gene is located, are among the most common genetic abnormalities in non-Hodgkin's lymphoma of B-cell type (B-NHL). The BCL6 gene encodes a BTB/POZ zinc finger transcription factor, which exerts repressive activity by recruiting corepressor molecules. The 3q27/BCL6 translocation is unique in that it can involve not only immunoglobulin (Ig) genes but also non-Ig chromosomal loci as a partner. To date, around 20 non-Ig partner genes have been identified. As a result of non-Ig ; BCL6 translocations, many types of regulatory sequences of each partner gene substitute for the 5' untranslated region of BCL6, and the rearranged BCL6 comes under the control of the replaced promoter. The introduction of non-Ig ; BCL6 constructs into transformed cells led to high-level Bcl-6 protein expression in the nucleus, while BCL6 mRNA levels in clinical materials of diffuse large B-cell lymphoma (DLBCL) with non-Ig ; BCL6 translocations were unexpectedly low. A comparative study suggested that non-Ig ; BCL6 translocation and a low level of BCL6 mRNA expression are concordant indicators of a poor clinical outcome in cases of DLBCL. The coexistence of a non-Ig ; BCL6 translocation with t(14 ; 18)(q32 ; q21) in a single clone did not significantly affect the clinical features of follicular lymphoma. The pathogenetic and clinical implications of non-Ig ; BCL6 translocations in B-NHL subtypes may not be identical to those of Ig ; BCL6.
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PMID:Pathogenetic and clinical implications of non-immunoglobulin ; BCL6 translocations in B-cell non-Hodgkin's lymphoma. 1714 54

PAG/Cbp is a transmembrane adaptor protein involved in proximal immune signaling. It is expressed in reactive germinal centers (GC) of secondary lymphatic follicles and related malignant lymphomas. We studied PAG/Cbp expression in GC-like and non-GC-like diffuse large B-cell lymphoma (DLBCL) subtypes. Seventy-three cases of DLBCL identified among 155 malignant lymphomas were classified as GC-like DLBCL (CD10+ or CD10-, bcl-6+, and MUM1-) and non-GC-like DLBCL (CD10-, MUM1+ or CD10-, bcl-6+, MUM1+). PAG/Cbp was detected by monoclonal antibody MEM-255 following routine immunohistochemical procedures. Thirty-five of 40 GC-like DLBCLs (88%) and 20 of 33 non-GC-like DLBCL cases (61%) expressed PAG/Cbp. Four of 12 bcl-6-negative non-GC-like DLBCL cases (33%) were PAG/Cbp positive, and only 4 of 20 bcl-6-positive non-GC-like DLBCL cases (25%) were PAG/CBP negative. All 37 FL and all 5 Burkitt's lymphomas (BL) expressed PAG/Cbp, whereas all 6 mantle cell lymphomas (MCL) and 4 of 5 chronic lymphocytic leukemias (CLL/SLL) were PAG/Cbp negative. PAG/Cbp is a reliable GC marker. Its expression correlates with GC-like DLBC phenotype in a significant majority of cases. It is typically absent in MCL and SLL/CLL.
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PMID:Expression of transmembrane adaptor protein PAG/Cbp in diffuse large B-cell lymphoma: immunohistochemical study of 73 cases. 1738 85

Follicular lymphoma (FL) is a neoplasm originating from germinal centre cells, corresponding to 25-40% of non-Hodgkin's lymphomas. Transformation into diffuse large B cell lymphoma (DLBCL) occurs in about one-third of cases. CD5 is expressed in B-chronic lymphoid leukaemia/small lymphocytic lymphoma and mantle cell lymphoma, but can rarely be expressed in conjunction with CD10 in well-documented cases of FL. In this report one case of grade 1 FL is described, which transformed into a DLBCL 6 months after initial diagnosis, with both tumours expressing CD5. In both specimens, neoplastic cells were strongly positive for CD20, CD79a, bcl-2, bcl-6 and CD5 in virtually all cells. CD10 was strongly positive in initial specimens and weakly positive in the DLBCL. Investigation using the PCR confirmed the derivation of the DLBCL from the FL as they presented the same immunoglobulin heavy chain gene rearrangement and the same BCL2-J(H) break point.
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PMID:CD5-positive diffuse large B cell lymphoma arising from a CD5-positive follicular lymphoma. 1751 19

Clinicopathological and immunophenotypical characteristics in 24 patients with diffuse large B-cell lymphoma (DLBCL) under 30 years of age in Osaka, Japan were examined, and the results compared to those of DLBCL patients aged over 40 years in Osaka and of young DLBCL patients in Western countries. The level of LDH and IPI score at initial diagnosis were significantly lower in young than older patients. The sex ratio (M:F) and age range (median) in the young and older groups were 1.18 and 11-30 (24.8) years and 1.59 and 42-87 (62.4) years, respectively. Extranodal presentation was higher in the young group (83.3% versus 60.0%, P < 0.05). Based on immunophenotyping with anti-CD10, bcl-6, and MUM1 antibodies, the cases were categorized as germinal center B-cell (GCB) (CD10+ or CD10-, bcl-6+, MUM1+) or non-GCB phenotype. The frequency of GCB type was significantly lower in the young group than older group (25% vs. 54%, P < 0.05), and much lower than that reported for young patients in Western countries. In situ hybridization revealed one of the young patients to be positive for Epstein-Barr virus (EBV). In the older group, none of 31 cases showed EBV positivity. Three year event-free and overall survival rates of young patients were better than those of the older patients, although not significantly different. DLBCL in the young in Japan is characterized by a much lower frequency of the GCB phenotype compared to that in Western countries.
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PMID:Diffuse large B-cell lymphoma in the young in Japan: a study by the Osaka Lymphoma Study Group. 1757 93

Primary testicular lymphomas typically occur in patients over 60 years of age. Most are diffuse large B-cell lymphomas with frequent dissemination and a poor prognosis. Primary follicular lymphoma of the adult testis has not been well characterized. However, a small number of primary testicular follicular lymphomas have recently been described in children. These showed stage 1E disease, a lack of BCL2 gene rearrangement and Bcl-2 protein expression, and a good clinical outcome. Here, we describe 5 cases of primary follicular lymphoma of the testis and epididymis in adults. These presented as unilateral testicular masses 12 to 40 mm in diameter and were characterized histologically by small neoplastic follicles in a sclerotic background. The neoplastic cells expressed CD10 and Bcl-6, but not Bcl-2 and lacked t(14;18)(q32;q21)/IGH-BCL2 and BCL6 gene rearrangements. Four of the five patients were 35 years old or younger, and 4 presented with stage 1EA disease. Although follow-up is 12 months or less in 2 of the 5 patients, to date each has followed an indolent clinical course. These features are different from those of most adult nodal follicular lymphomas but are very similar to those of the pediatric primary testicular follicular lymphomas. Together, the pediatric and adult cases represent a discrete clinicopathologic entity of t(14;18)(q32;q21)/IGH-BCL2-negative primary follicular lymphoma of the testis and epididymis, which typically present as clinically indolent localized disease in young males and should be distinguished from the diffuse large B-cell lymphoma more frequently seen in the testes of older adults.
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PMID:Primary follicular lymphoma of the testis and epididymis in adults. 1759 72

Expression of neural cell-adhesion molecule CD56 is a rare event in B-cell lymphoma. We described four cases of CD56-positive B-cell lymphoma, including follicular lymphoma and diffuse large B-cell lymphoma. These lymphoma cells expressed CD10 and bcl-6, which suggests germinal center-stage phenotype. Immunohistochemistry showed that CD56-positive cells aggregated and displayed a cohesive growth pattern, indicating that homotypic adhesion through the molecules might affect the manner of tumor growth and expansion. Although CD56 expression level varies among the cases, this molecule might play some roles in the manner of growth and expansion of CD56-positive B-cell lymphomas.
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PMID:Neural cell adhesion molecule (CD56)-positive B-cell lymphoma. 1763 42


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