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)

A 52-year-old man, who had been diagnosed as having alcoholic liver cirrhosis, presented a chronic nephritic syndrome due to hepatic glomerulosclerosis. Ten months before death, massive proteinuria exceeding 40 g/day was noted. A renal biopsy revealed diffuse mesangial sclerosis, associated with an IgA deposition consistent with hepatic glomerulosclerosis. Although the nephrotic syndrome subsided with immunosuppressive therapy, he died of hepatic failure. Postmortem examinations disclosed a diffuse, medium-size B-cell lymphoma, involving the peritoneal and retroperitoneal organs and an IgA-positive plasmacytosis. His massive proteinuria seems to have been caused by the paraneoplastic syndrome of a malignant lymphoma.
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PMID:[An autopsied case of a malignant lymphoma with a severe nephrotic syndrome overlapped by cirrhotic glomerulosclerosis]. 329 70

We report a case of smoldering adult T-cell leukemia (ATL) with B-cell lymphoma and early gastric cancer. A 64-year-old man was admitted to our hospital because of proteinuria and hypergammaglobulinemia. Systemic lymphadenopathy, "flower cells" in peripheral white blood cells, and hypergammaglobulinemia with monoclonal gammopathy (IgA, lambda type) were found. As Southern blot analysis revealed monoclonal integration of human T-lymphotrophic virus type I proviral DNA in peripheral blood mononuclear cells, he was diagnosed as having smoldering ATL. The tissue specimen of an inguinal lymph node showed proliferation of abnormal lymphocytes which were stained with anti-lambda antibody, indicating B-cell lymphoma. A polypoid lesion in the stomach was histologically diagnosed as early gastric cancer.
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PMID:Smoldering adult T-cell leukemia with B-cell lymphoma and early gastric cancer. 772 76

Kidney and the urogenital tract are among the various mucosal sites involved in mucosa-associated lymphoid tissue (MALT) lymphoma. We report a case with simultaneous onset of crescentic immunoglobulin (Ig) A nephropathy and gastrointestinal low-grade B-cell lymphoma of the MALT type with kidney infiltration. M-component of IgM lambda was detected in the serum, and the renal biopsy specimen showed monotypic lambda light chain staining in the lymphoma cells but not the glomeruli. The heavy proteinuria and impaired creatinine clearance returned to normal, and microscopic hematuria disappeared 20 months after treatment with chlorambucil as single-agent chemotherapy. This coincided with a complete resolution of the gastric and renal lymphoma infiltration. The close association of both the onset and successful outcome of the two entities thus support their possible causal relationship, and we discuss the possibility of an association of the disturbance of the MALT by the lymphoma cells with the pathogenesis of IgA nephropathy.
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PMID:Successful treatment of IgA nephropathy in association with low-grade B-cell lymphoma of the mucosa-associated lymphoid tissue type. 953 Nov 92

(NZB x NZW)F1 female (BW) mice spontaneously develop an autoimmune disease, characterized by the production of autoantibodies (autoAbs) and glomerulonephritis, which can be delayed by neutralizing IFN-gamma Abs and accelerated by IFN-gamma injections. To define the role of IFN-gamma in the pathogenesis of glomerulonephritis, we established a population of BW mice deficient in IFN-gammaR (BWgammaR[-/-]) by repeated crossing; these mice were compared with BWgammaR(+/+) and +/- littermates. Of the BWgammaR(+/+) and +/- mice, 50% showed immune complex glomerulonephritis with heavy proteinuria at 8 mo of age, while only 10% of the BWgammaR(-/-) mice were affected at 14 mo. The serum concentration of anti-dsDNA and anti-histone Abs was dramatically reduced in BWgammaR(-/-) mice. The role of IFN-gamma in promoting class switch to IgG2a and IgG3 could not fully account for the impaired production of anti-dsDNA in BWgammaR(-/-) animals since, IgM and IgG1 levels were also reduced. There was a high incidence of B cell lymphoma in the BWgammaR(-/-) mice, which might be related to the suppression of autoAb production. Thus, the absence of glomerulonephritis in BWgammaR(-/-) mice is likely due to a dramatic yet unexplained effect of the inactivation of IFN-gamma signaling on autoAb production.
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PMID:IFN-gamma receptor deletion prevents autoantibody production and glomerulonephritis in lupus-prone (NZB x NZW)F1 mice. 955 72

Low-grade B cell lymphoma of mucosa-associated tissue type (MALToma) rarely may involve the kidney. Membranoproliferative glomerulonephritis (MPGN) is an uncommon complication of B cell lymphoma and may be related to cryoglobulin and/or immunoglobulin synthesis by a secretory B cell clone. We report 2 patients with the novel renal biopsy findings of coexistent MALToma and MPGN. Both subjects presented with nephrotic proteinuria and renal insufficiency. One patient had a serum M protein (IgG K) but neither individual had any other clinical or serologic evidence of systemic disease, including hematolymphoid malignancy, autoimmune disease, cryoglobulinemia, or hepatitis C viral infection. Both renal biopsies demonstrated MPGN type I with immunoglobulin deposits that in 1 case showed light chain restriction (IgM K). Electron microscopy disclosed corresponding glomerular electron dense deposits in subendothelial locations. Both biopsies also contained atypical interstitial lymphoid infiltrates comprising marginal zone (centro-cyte-like) cells that infiltrated tubules and showed extra-capsular extension. Immunostains demonstrated a predominantly B cell population that lacked expression of CD5 and cycline D1, and gene rearrangement studies confirmed the presence of a monoclonal B cell population in both cases. These findings indicate that low-grade B cell lymphoma in the kidney may be an unexpected finding in patients with nephrotic syndrome related to MPGN. Immunophenotypic and gene rearrangement studies are important ancillary tools for the evaluation of atypical lymphoid infiltrates in kidney biopsies.
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PMID:Membranoproliferative glomerulonephritis associated with low-grade B cell lymphoma presenting in the kidney. 1200 47

We report 39 years old man with the history of chronic sinusitis and rhinitis. After tooth extraction he gradually developed unilateral proptosis with ophtalmoplegia and visual loss caused by retroorbital mass which was related to destruction of the adjacent orbital walls, sinuses and base of the skull. During the following month the progressing lung nodules with mediastinal and hilar lymphadenopathy, macular skin lesions, renal insufficiency with proteinuria and anaemia appeared. The diagnosis of Wegener's Granulomatosis (WG) was formed on the base of clinical features and result of pathologic examination of surgical specimen from the paranasal sinuses. The progressive course under the standard immunosuppressive therapy required reevaluation of histologic slides, which resulted with the diagnosis of diffuse large B-cell lymphoma confirmed by the immunohistochemical staining. Administration of CHOP regimen resulted in spectacular regression of all of lesions.
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PMID:[Diffuse large B-cell lymphoma mimicking Wegener's granulomatosis]. 1227 68

We report the case of an intravascular large B-cell lymphoma in a 49-year-old woman, which was revealed by proteinuria. This type of lymphoma is very rare and corresponds to the proliferation of malignant lymphoid cells within the lumina of small vessels, resulting in ischemic lesions involving mainly brain and skin. Renal involvement is quite rare and remains asymptomatic, being discovered at autopsy. In this case, the renal biopsy demonstrated the presence of large malignant B cells in the glomerular lumina, associated with minimal glomerular damage. The mechanisms of the proteinuria occurring during intravascular lymphoma remain to elucidate.
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PMID:[Intravascular large B-cell lymphoma revealed by proteinuria: a case report]. 1241 Jan 7

A 12-year-old Japanese boy who underwent kidney transplantation with a kidney from his mother developed severe proteinuria immediately after the operation. Because his original disease was nephrotic syndrome (focal segmental glomerulosclerosis, or FSGS) and electron microscopic examination of the renal biopsy showed foot process fusion, we diagnosed this as a recurrence of nephrotic syndrome to the transplanted kidney. Four months after the transplantation, posttransplant lymphoproliferative disorder (PTLD) developed, which was pathologically diagnosed as diffuse large B cell lymphoma. Treatment consisting of a reduction in immunosuppression resulted in improvement in PTLD a month after the start of treatment. However, relapse occurred 2 months after the first onset of PTLD, which we treated with rituximab (CD-20 monoclonal antibody 375 mg/m2) once weekly for a total of four doses. The PTLD resolved immediately after the rituximab treatment was started, and, interestingly, urinary protein levels also improved at the same time. Three years later, the boy shows no signs of PTLD, and no proteinuria has been detected. These findings suggest that rituximab may be an effective treatment for recurrence of nephrotic syndrome after transplantation and that activated B cells may play a pivotal role in the recurrence of nephrosis after renal transplantation.
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PMID:Rituximab treatment for posttransplant lymphoproliferative disorder (PTLD) induces complete remission of recurrent nephrotic syndrome. 1693 30

We report a 35-year-old Japanese woman with intravascular large B-cell lymphoma diagnosed by percutaneous renal biopsy. The patient was referred to our institution for further examination of fever of unknown origin. She had renal dysfunction with a creatinine clearance of 44.1 mL/min, and daily urinary excretion of 0.22 g of protein and 21.5 mg of beta 2 microglobulin. Computed tomography showed markedly enlarged kidneys bilaterally. Percutaneous renal biopsy showed that an island-like atypical lymphoid cell accumulation was encircled with the peritubular capillary walls in many areas of the tubulo-interstitium, resulting in marked destruction of tubular structure. However, almost all the glomeruli were intact. Immunohistochemical analysis confirmed the diagnosis of intravascular large B-cell lymphoma. Shortly after diagnosis, she was treated with rituximab, cyclophosphamide, hydroxydaunomycin, oncovin, and prednisolone, and her renal function and size improved. Renal involvement by lymphoma has been classified into two categories: intraglomerular intravascular lymphoma and tubulointerstitial diffuse invasion type that is distinct from intravascular lymphoma. For the latter cases with renal dysfunction and marked bilateral nephromegaly but without proteinuria, intravascular lymphoma within intra-peritublar capillaries should be considered as a possible diagnosis.
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PMID:Renal intravascular large B-cell lymphoma localized only within peritubular capillaries. Report of a case. 1752 39

In a 56-year-old white male patient, a membranoproliferative glomerulonephritis Type I was diagnosed after a 12-month history of low grade B cell lymphoma (Binet A). HIV, Hepatitis B and C serology were negative. Due to an impairment of renal function despite chemotherapy with COP, an immunochemotherapy consisting of rituximab (6 cycles) and bendamustine (4 cycles) was given. This therapeutic approach caused a complete remission of the nephrotic syndrome. Renal function and arterial hypertension improved markedly. In addition, urinary sediment became normal and proteinuria disappeared completely.
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PMID:Remission of a B cell CLL-associated membranoproliferative glomerulonephritis Type I with rituximab and bendamustine. 1839 3


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