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)

In the World Health Organization classification system, splenic marginal-zone lymphoma (splenic MZL) is described as an indolent B-cell lymphoma, which generally presents as splenomegaly with involvement of the bone marrow and peripheral blood. Presence of disease in peripheral lymph nodes and extranodal locations is uncommon. Splenic MZL is characterised by micronodular infiltration of the spleen with marginal-zone differentiation; the immunophenotype is usually IgM+ IgD+/- cytoplasmic-Ig-/+ pan B antigens+ CD5- CD10- CD23- CD43-/+ cyclin D1-; and the most common genetic abnormalities are deletions at 7q22-7q32. Most patients with splenic MZL live for a long time but classic prognostic factors cannot distinguish between patients who are likely to have good and poor outcomes. However, immunological events, such as haemolytic anaemia and immune thrombocytopenia, or the presence of a monoclonal component, are significantly associated with shorter survival. Splenectomy is considered the first-line treatment of choice for splenic MZL; it results in only partial remission, but responses are generally sufficient for correcting cytopenia, improving quality of life, and increasing survival.
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PMID:Splenic marginal-zone lymphoma: a distinct clinical and pathological entity. 1257 51

Coexpression of CD5 and CD10 is highly unusual in B-cell lymphomas and may pose a diagnostic challenge. We report 42 cases of B-cell lymphoma with simultaneous expression of CD5 and CD10. They made up approximately 0.4% of all B-cell lymphomas seen during the study period and included the following cases: large B-cell lymphoma (LBCL), 14 (33%); follicular lymphoma (FL), 10 (24%); mantle cell lymphoma (MCL), 9 (21%); chronic lymphocytic leukemia, 4 (10%); acute precursor B-cell lymphoblastic leukemia/lymphoma, 2 (5%); and other low-grade B-cell lymphomas, 3 (7%). All MCLs had overexpression of bcl-1 or the t(11;14) and were CD43+. All FLs had typical histomorphologic features and were bcl-2+ and bcl-6+ but CD43-. Of 14 LBCLs, 5 were histologically high-grade. Six (43%) of 14 patients with LBCL died within 10 months of diagnosis of CD5+CD10+ lymphoma (median survival, 4 months), including all 3 patients with stage IV disease and 2 of 5 with histologically high-grade lymphoma. Our findings indicate that coexpression of CD5 and CD10 is rare but occurs in diverse subtypes of B-cell lymphoma. Investigation of bcl-1, bcl-6, and CD43 and morphologic evaluation may resolve the potential confusion in diagnosis and lead to the recognition of the correct lymphoma subtype.
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PMID:B-cell lymphomas with coexpression of CD5 and CD10. 1257 92

Crystal-storing histiocytosis (CSH) is a rare disorder occurring in patients with lymphoproliferative diseases, predominantly in cases of multiple myeloma and low-grade B-cell lymphoma. To the best of our knowledge, only three cases of pulmonary CSH have been reported in the English literature and one of them was associated with a low-grade B-cell lymphoma (immunocytoma). We document a case of a 59-year-old man with bilateral lung masses in which a right middle lobe pulmonary lobectomy specimen showed CSH associated with an extranodal marginal-zone B-cell lymphoma. A single nodule showing features of fibroleiomyomatous hamartoma was present in a wedge biopsy specimen from the left lung. Two nodules within the right middle lobe were composed of sheets of histiocytic cells with abundant eosinophilic cytoplasm resembling striated muscle cells. In addition, there were nodular aggregates and a more diffuse infiltrate of small slightly atypical centrocyte-like lymphocytes, as well as bronchial lymphoepithelial lesions. Immunohistochemistry performed on paraffin-embedded sections demonstrated that the histiocytic cells were immunoreactive with the KP-1 (CD68) antibody while the lymphocytic infiltrate was CD20 positive, co-expressed for CD43, and was negative for CD3, CD5, and CD10. Genotypic analysis demonstrated the presence of an immunoglobulin heavy-chain gene rearrangement, indicating the presence of a monoclonal B-cell population. These features were consistent with pulmonary CSH associated with extranodal marginal-zone lymphoma of baltoma type.
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PMID:Pulmonary crystal-storing histiocytosis and extranodal marginal zone B-cell lymphoma associated with a fibroleiomyomatous hamartoma. 1261 74

A unique, previously unreported case of transformation of cutaneous plasmacytoma into CD30+ large B-cell lymphoma is described. Both neoplastic components were immunophenotypically distinct. The plasma cells were CD20-, CD30-, CD43+, CD45+, lambda +; the blasts were CD20+, CD30+, CD43-, and CD45-. The large B-cell lymphoma has gradually become a predominant component of the neoplastic nodules. While plasma cells and blasts were both positive for Epstein-Barr virus-encoded nuclear RNAs (EBER-1), the EBV-latent membrane antigen 1 (EBV-LMP1) was expressed only in the minority of the blasts and not in the plasma cells. The neoplastic process has remained confined to the skin for more than six years since its development.
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PMID:Epstein-Barr virus-induced transformation of cutaneous plasmacytoma into CD30+ diffuse large B-cell lymphoma. 1472 24

A 47-year-old Hispanic male presented with visual field disturbances, memory impairment, and a seizure. CT and MRI were consistent with meningioma. Both neurologic exam and routine laboratory tests were within normal limits. The patient underwent craniotomy and subtotal resection of the tumor. On H&E, the lesion was composed of a lymphoid mass with well-defined irregularly shaped follicles surrounded by a monomorphic population of small lymphocytes. Marginal zones stained for B-cell markers, CD20 and CD79a, one T-cell marker, CD43, and kappa light chains. While other markers did not stain the majority of tumor cells, they did identify other lymphoid and plasma cell elements. A diagnosis of marginal zone B-cell lymphoma of dura, mucosa-associated lymphoid tissue (MALT)-type (extranodal) was made. MALT-type lymphomas are unusual in the nervous system; this is the first such case reported in a male and serves to emphasize the wide diversity of presentation of a neoplasm originally described in the GI tract and thus far described in the CNS only in females.
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PMID:Primary dural mucosa-associated lymphoid tissue-type lymphoma: case report and review of the literature. 1517 17

B cells are divided into two categories: conventional or B-2B cells and B-1B cells, the latter of which are distinguished by their different ontogeny. B cell lymphoma 1 (BCL1), the first-reported case of a spontaneously developed mouse B-lymphoma, expresses CD5, surface IgM, Mac-1, CD43 and low level of B220, and is likely to have B-1a cell origin. However, antigens recognized by IgM produced by the BCL1 cells (BCL1-IgM) have not been identified. Here, we demonstrate that BCL1-IgM reacts with Escherichia coli (E. coli). Our initial finding that several recombinant proteins expressed in E. coli bound to BCL1-B20 prompted us to examine the possibility that BCL1 cells may bind E. coli. Indeed, BCL1 cells bound fluorescein-labeled E. coli. To elucidate the structure on the BCL1 cells responsible for E. coli-binding, we produced a monoclonal antibody capable of inhibiting BCL1 binding to E. coli. The antibody recognizes an idiotypic epitope on the BCL1-IgM. Moreover, polyclonal antibody against IgM and secreted BCL1-IgM purified from the supernatants inhibited BCL1 binding to E. coli. Finally, transfection of non-lymphoid cells with cDNA of heavy and light chains of BCL1-IgM conferred the cells ability to bind E. coli. These results clearly indicate that BCL1-IgM bind E. coli and suggest that BCL1 lymphoma is a typical B-1 cell-derived lymphoma, characterized not only by the surface phenotype, but also by the reactivity of its IgM with commensal bacteria.
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PMID:B-1a cell origin of the murine B lymphoma line BCL1 characterized by surface markers and bacterial reactivity of its surface IgM. 1586 Feb 23

Expression of CD43 by B cells is often used as a diagnostic criterion in favor of a B-cell lymphoproliferative disorder, including small lymphocytic lymphoma/chronic lymphocytic leukemia, mantle cell lymphoma, Burkitt lymphoma, precursor B-lymphoblastic lymphoma, and a subset of marginal zone B-cell lymphomas. Benign B cells generally do not coexpress CD43. The authors analyzed 20 biopsies of the terminal ileum for nonneoplastic disease for expression of CD43 and compared them with other sites and with CD20, CD138, and CD3 reactivity. The majority of cases (85%) showed strong coexpression of CD43 by benign perifollicular B cells. The presence of CD43 coexpression in B-cell populations of the terminal ileum, including those of Peyer's patches, should not be used as a diagnostic parameter to differentiate extranodal marginal zone B-cell lymphoma of MALT type from reactive processes.
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PMID:Coexpression of CD43 by benign B cells in the terminal ileum. 1589 25

To clarify the confusion surrounding the diagnosis of cutaneous lymphoid hyperplasia (CLH) that was formerly described as lymphadenosis benigna cutis, lymphocytoma cutis, or lymphocytic infiltration of Jessner and to assess whether newly recognized diagnoses, such as cutaneous marginal zone lymphoma and pseudolymphomatous folliculitis (PLF), may have been overlooked, we reexamined 55 Japanese cases of nonepidermotropic lymphoproliferative disorder that had previously been diagnosed as "cutaneous pseudolymphoma." In all these cases, the immunohistochemical expressions of CD1a, CD3, CD4, CD8, CD20, CD21, CD30, CD43, CD56, CD68, CD79a, kappa and lambda chains, S-100 protein, and latent membrane protein were assessed. In addition, in 13 cases the gene rearrangement of the immunoglobulin heavy chain was investigated using a polymerase chain reaction method. As a result of these investigations, we have identified 4 cases of cutaneous marginal zone lymphoma, 19 cases of PLF, 1 case of diffuse large B-cell lymphoma, and 2 cases of solitary nonepidermotropic pseudo-T-cell lymphoma, with the remaining 29 cases being CLH. Cutaneous marginal zone lymphoma, which represented 7.3% of the total, was distinguished from CLH by the presence of patchy or diffuse proliferation of centrocyte-like cells, plasma cells at the periphery of the lymphocytic infiltration, monotypic restriction of the light chains, and gene rearrangement of the immunoglobulin heavy chain. Pseudolymphomatous folliculitis was identified by the presence of activated pilosebaceous units with abundant CD1a-and S-100 protein-positive T-cell-activated dendritic cells. Of the cases that were reassessed, 34.5% were PLF.
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PMID:A review of 55 cases of cutaneous lymphoid hyperplasia: reassessment of the histopathologic findings leading to reclassification of 4 lesions as cutaneous marginal zone lymphoma and 19 as pseudolymphomatous folliculitis. 1594 17

We report the case of a 79-year-old woman with a longstanding lymphedema of the right arm who developed a skin lymphoma involving the right wrist area. Microscopically, the lesion was composed of numerous centroblasts infiltrating both the dermis and the subcutaneous tissue. Phenotypic investigations showed expression of CD20, CD79a, and bcl-2 protein by neoplastic cells. In addition, these cells were CD5 positive. No expression of anaplastic large cell lymphoma kinase (ALK), CD10, CD23, CD30, CD43, bcl-6, cyclin D1, p53 or p16INK4a could be seen. Polymerase chain reaction (PCR) analysis demonstrated a clonal rearrangement of the genes coding for the kappa light chain of the immunoglobulin (Ig). No rearrangement of the genes coding for the Ig heavy chain, t(14;18) or t(11;14) chromosome translocations, or Epstein-Barr virus (EBV) genomic sequences could be found. The tumor was classified as stage IE and was first cured by complete surgical excision. Nineteen months later, a recurrence was noted in the right elbow area. This study further illustrates that lymphoma of the skin may complicate chronic limb lymphedema. Like most of the previously reported cases, this neoplasm belonged to the category of diffuse large B-cell lymphoma. However, it showed CD5 expression as a singular feature.
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PMID:De novo CD5-positive diffuse large B-cell lymphoma of the skin arising in chronic limb lymphedema. 1601 18

Pyothorax-associated lymphoma (PAL) is a B-cell lymphoma which develops in the pleural cavity of patients with an over-20-year history of pyothorax. Aberrant expression of surface antigens is occasional in PAL, although genotype is not fully investigated. We report here a PAL with dual genotype, i.e., simultaneous immunoglobin (Ig) and T-cell receptor (TcR) gene rearrangement. An 82-year-old woman with pain on the left side of the chest was admitted. She had been suffering from pyothorax after artificial pneumothorax for treatment of tuberculosis of the pulmonary when she was 18 years old. The mass that was confined to the left pleural cavity affected by pyothorax was biopsied and histologically diagnosed as diffuse large cell lymphoma. The tumor cells were positive for CD20, CD16, and TIA-1 but negative for CD79a, CD45RO, CD43, CD3, and CD56. Surface antigen expression was further investigated in cultured cells, showing that the cultured cells did not express representative B-cell markers, except for CD20, as well as T-cell markers, but were positive for CD16, CD30, and CD103. Southern blotting revealed the monoclonally rearranged bands of both Ig heavy chain and TcR gene. The patients died of tumors 14 months after admission. Aberrant genotype and immunophenotype of PAL cells is discussed in reviewing the pertinent literature.
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PMID:Pyothorax-associated lymphoma: an unusual case with both T- and B-cell genotypes. 1602 7


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