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)

Expression patterns of eight transcription factors involved in different stages of B-cell development were investigated in a large group of primary cutaneous B-cell lymphomas and compared with expression patterns during normal B-cell development. The following transcription factors were investigated: Pax-5, PU.1, Oct2, BOB.1, Bcl-6, Mum1/IRF4, Blimp-1 and FOXP1. Primary cutaneous large B-cell lymphomas, leg type showed aberrant coexpression of Bcl-6 and Mum1/IRF4 and in addition strong expression of FOXP1. Expression of FOXP1 and Mum1/IRF4 strongly suggests an activated B-cell type of origin. In contrast, primary cutaneous follicle center lymphomas showed expression of Bcl-6, Pax-5, PU.1, Oct2 and BOB.1, but not of Mum1/IRF4, Blimp-1 and FOXP1. Primary cutaneous marginal zone B-cell lymphoma showed expression of Pax-5, PU.1, Oct2 and BOB.1, but not Bcl-6 by the neoplastic B-cells, and Mum1/IRF4 and Blimp-1 by the neoplastic plasma cells. In conclusion, in primary cutaneous follicle center lymphoma and primary cutaneous marginal zone B-cell lymphoma expression patterns were observed similar to their supposed benign counterparts, germinal center B-cells and postgerminal center B-cells, respectively, which might reflect their indolent clinical behaviour and excellent prognosis. In contrast, the activated B-cell expression pattern in the group of primary cutaneous large B-cell lymphoma, leg type may contribute to its poor prognosis and Mum1/IRF4 and FOXP1 may serve as additional diagnostic markers for this type of primary cutaneous B-cell lymphoma.
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PMID:Expression of B-cell transcription factors in primary cutaneous B-cell lymphoma. 1677 25

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

Primary cutaneous marginal zone B-cell lymphoma (PCMZL) is a recently proposed entity and constitutes the cutaneous counterpart of extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT). The t(14;18)(q32;q21) involving the IGH and the MALT1 gene has previously been described in PCMZL, whereas the t(14;18)(q32;q21) IGH/BCL2 seems to be restricted to follicular lymphoma and diffuse large B-cell lymphoma. We screened 30 PCMZLs of 13 patients by fluorescent in situ hybridization analysis for the presence of the t(14;18)(q32;q21) IGH/BCL2 and the t(14;18)(q32;q21)IGH/MALT1. The t(14;18)(q32;q21) IGH/MALT1 was detected in 10 PCMZLs of eight patients, with four patients showing the t(14;18)(q32;q21) IGH/MALT1 exclusively. The t(14;18)(q32;q21)IGH/BCL2 was detected in 16 PCMZLs of seven patients, with four patients showing the t(14;18)(q32;q21) IGH/BCL2 exclusively. Six lymphomas of four patients showed both translocations in the same lesion. In seven lymphomas, neither of the two translocations occurred. One patient developed multiple lesions without either of the two translocations. Our results underline that both the t(14;18)(q32;q21)IGH/BCL2 and the t(14;18)(q32;q21) IGH/MALT1 may occur in PCMZL, albeit in an irregular distribution. Therefore, the etiopathogenetic relevance of either translocation in PCMZL remains a matter of debate.
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PMID:Primary cutaneous marginal zone B-cell lymphoma may exhibit both the t(14;18)(q32;q21) IGH/BCL2 and the t(14;18)(q32;q21) IGH/MALT1 translocation: an indicator for clonal transformation towards higher-grade B-cell lymphoma? 1751 19

Primary cutaneous marginal zone B-cell lymphoma is considered the cutaneous counterpart of extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue. Although its molecular pathogenesis is currently unknown, an etiological link with Borrelia burgdorferi infection has been identified in European, but not in American or Asian cases. To better understand the pathogenesis and the geographical differences of cutaneous marginal zone B-cell lymphoma, 60 cases from the East Asia, Germany, and the United States at their initial presentation were subjected to the following analyses; (1) clinicopathological comparison between the geographical regions, (2) detection of B. burgdorferi DNA, (3) detection of the API2-MALT1 fusion transcript, a gene alteration specific to mucosa-associated lymphoid tissue lymphoma, and (4) inactivation of tumor suppressor genes (death-associated protein kinase (DAPK), p16(INK4a), p14(ARF), MGMT, TIMP3, CDH1, and RARB) by hypermethylation of the CpG islands. Cases from the three geographical regions showed similar clinicopathological features. However, moderate/marked tissue eosinophilia was found in 9/25 Asian cases, but only 1/23 German cases (P=0.011) and 0/12 American cases (P=0.015). All 60 cases were negative for either Borrelia DNA or API2-MALT1 fusion. Tumors from the three regions were highly methylated for DAPK (38-50% of the cases, mean 43%) and p16(INK4a) (42-70%, mean 49%), and the positivities were significantly higher than those of nonneoplastic skin (8%, P=0.0010 and 14%, P=0.0032, respectively). Methylation of these genes had no significant association with progressive features of the tumor. Primary cutaneous marginal zone B-cell lymphomas from the three geographical regions have common clinicopathological features, however, moderate/marked tissue eosinophilia is a feature found almost exclusively in Asian cases. Borrelia infection and API2-MALT1 fusion are not significant in this tumor. Methylation of DAPK and p16(INK4a) genes is a frequent event in this lymphoma at its initial presentation, but may not be associated with tumor progression.
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PMID:Primary cutaneous marginal zone B-cell lymphoma: a molecular and clinicopathological study of cases from Asia, Germany, and the United States. 1882 Jun 62

Primary cutaneous marginal zone B-cell lymphoma (PCMZL) is an indolent low grade B cell lymphoma of the skin, with lack of extracutaneous involvement at the time of diagnosis. Herein we report the case of a patient with rheumatoid arthritis (RA) who developed symmetrical PCMZL lesions on both ear lobes. Lesions occurring symmetrically on ear lobes are more specific for cutaneous lymphoid hyperplasia (CLH) and this kind of symmetrical localization hasn't been reported for PCMZL before. PCMZL is considered to arise from a background of reactive lymphoid hyperplasia and this case point out the concept of CLH and PCMZL spectrum. Association of marginal zone lymphoma with rheumatoid arthritis and resolution of lesions together with the resolution of symptoms due to rheumatoid arthritis after rituximab therapy is another interesting point for this case. To the best of our knowledge PCMZL associated with RA has not been reported previously.
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PMID:Symmetrical primary cutaneous marginal zone lymphoma associated with rheumatoid arthritis. 1967 36

According to the WHO-EORTC classification of cutaneous lymphomas, primary cutaneous marginal zone B-cell lymphoma are now well characterized. We report here a case of primary cutaneous marginal zone B-cell lymphoma in a 51 year-old man in which the diagnosis was made using both histology and immunopathology. The patient had no remarkable medical history, no history of either acute inflammation or insect bite, and presented with a 5 cm solitary asymptomatic erythematous firm, multinodular and infiltrated plaque on the back for 12 months. Histological examination and immunohistochemical study of a cutaneous biopsy provided a differential diagnosis between B cell lymphoma and lymphocytoma cutis. Full body work up revealed no signs of extracutaneous dissemination. The patient underwent surgical excision of the nodule. Histological examination showed a histological and immunophenotyping profile typical of primary cutaneous marginal zone B-cell lymphoma. The lesion was completely excised with clear margins and no recurrence occurred after a 12 month-follow-up period. Primary cutaneous marginal zone B-cell lymphoma are low-grade lymphomas that have an indolent course and a high tendency to recur. They should be differentiated from lymphocytoma cutis and from the other types of cutaneous B cell lymphomas that have a different course and prognosis.
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PMID:Primary cutaneous marginal zone B-cell lymphoma: clinical and histological aspects. 1977 68

Primary cutaneous marginal zone B-cell lymphoma (PCMZL) is included in the group of extranodal marginal zone B-cell lymphoma involving mucosal sites. Many evidences suggest that chronic antigen stimulation is a key-player in its pathogenesis. While Helicobacter pylori seems not to be implicated in PCMZL, Borrelia Burgdorferi's role is still matter of debate since the results are discordant between European and North American/Asian countries. However Borrelia subspecies are different between the studied areas and this difference could be a confounding factor. Then ubiquitous candidate antigen is still missing. Beyond these discrepancies the treatment of diffuse PCMZL has been recently improved. If local therapies (surgery, radiation) are the gold standard for localized disease, rituximab can also be considered as an alternative for disseminated or plurifocal PCMZL.
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PMID:Primary cutaneous marginal zone lymphoma. 1981 17