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)

Peripheral neuropathy associated with IgM monoclonal gammopathy of unknown significance is a common disorder, while the association of paraproteinaemic neuropathies with haematological malignancies is far less frequent. We report a 76-year-old patient with a subacute and rapidly progressive sensorimotor demyelinating polyneuropathy causing sensory ataxia, painful paraesthesias and marked motor and sensory deficit in four limbs. Monoclonal gammopathy of IgM type associated with a rectal low-grade B-cell non-Hodgkin lymphoma was detected. Research for anti-MAG and antiganglioside autoantibodies including anti-GM1 and anti-GQ1b evidenced a high titre of IgM antibodies against the disialosyl group of GD1b. This is the first report on a paraproteinaemic polyneuropathy with IgM autoantibodies against glycolipid GD1b associated with B-cell lymphoma. The IgM type of these autoantibodies suggests that they represent all or part of the paraprotein produced by lymphoma cells.
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PMID:Subacute demyelinating polyneuropathy in B-cell lymphoma with IgM antibodies against glycolipid GD1b. 1638 73

Monoclonal gammopathy is characterized by circulating monoclonal immunoglobulin owing to clonal proliferation of immunoglobulin-producing B lymphocytes or plasma cells. Clonal proliferation of B lymphocytes is seen in B-cell lymphoma/leukemia, and clonal plasma cell proliferation is seen in multiple myeloma and monoclonal gammopathy of undetermined significance. The monoclonal immunoglobulin in the setting of a B-cell or plasma cell disorder can cause a proliferative glomerulonephritis via 2 mechanisms: (1) glomerular deposition of the monoclonal immunoglobulin with activation of the classical pathway of complement (direct mechanism), resulting in an immunoglobulin-positive C3-positive glomerulonephritis, and (2) glomerular deposition of complement factors of the alternative and terminal pathway via inhibition of alternative pathway-regulating proteins by the monoclonal immunoglobulin (indirect mechanism), resulting in immunoglobulin-negative C3-positive glomerulonephritis (C3 glomerulopathy). Evaluation should include serum and urine electrophoresis and immunofixation as well as serum-free light-chain assay. If a monoclonal immunoglobulin is detected on these tests, bone marrow biopsy or imaging is needed to exclude more advanced plasma cell dyscrasia. Evaluation of alternative pathway of complement should be done in patients with Ig-negative C3-positive glomerulonephritis. If monoclonal gammopathy is due to an underlying malignant disease such as myeloma, lymphoma, or chronic lymphocytic leukemia, then specific treatment should be aimed at treating the malignant disease, with the goal of eradicating the clonal cells producing the immunoglobulin. In contrast, if monoclonal gammopathy is due to a monoclonal gammopathy of undetermined significance, treatment options include bortezomib, cyclophosphamide, and dexamethasone for a non-IgM monoclonal immunoglobulin and rituximab alone or in combination with cyclophosphamide and dexamethasone for an IgM monoclonal immunoglobulin.
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PMID:Monoclonal gammopathy-associated proliferative glomerulonephritis. 2418 5

Monoclonal gammopathy (MG), a positive result of serum immunofixation electrophoresis (SIFE), has been reported in cases of diffuse large B-cell lymphoma (DLBCL). We performed this study to further investigate the prognostic value of MG in DLBCL.We retrospectively reviewed patients diagnosed with DLBCL between January 2007 and December 2014, and identified 37 patients with MG. The clinical characteristics of these patients were then reviewed. A 1:2 case-control analysis was conducted on 74 matched controls, who were patients with DLBCL and without MG. Both cases and controls were age-matched and were diagnosed within the same year.Among 37 DLBCL patients with MG, the monoclonal component of IgM was the most frequent compared to the other subtypes. Laboratory tests showed that the presence of MG was correlated with a decreased platelet-to-lymphocyte ratio (PLR). Survival analysis showed that MG-secreting DLBCL patients had an inferior overall survival (OS) and progression-free survival (PFS), compared with MG-nonsecreting patients, regardless of MG subtype. However, treatment response analysis showed that MG was not a good indicator for tumor relapse. When patients with DLBCL were grouped by immunophenotype, we found that MG was associated with poor prognosis in the non-germinal center B-cell-like (GCB) type, rather than GCB type in OS analysis. Meanwhile, there was no statistical significance upon PFS analysis in both immunophenotypes. Furthermore, our study found that the appearance of MG during treatment did make prognostic sense compared to nonsecretors.Overall, MG can serve as a prognostic factor for DLBCL. We hypothesize that its presence in DLBCL may reflect the immune microenvironment in tumor progression and warrants further study to unveil the underlying molecular pathogenesis.
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PMID:Monoclonal gammopathies regardless of subtypes are associated with poor prognosis of diffuse large B-cell lymphoma: A STROBE-compliant article. 3004 37