Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0026764 (multiple myeloma)
 36,148 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors describe a case which demonstrates that nephrotic syndromes occuring during the course of a myeloma are not always of the amyloid type. They emphasize the possibility of a dysglobulinemia being involved in a glomerular nephropathy syndrome, especially when there are histological lesions of a glomerulosclerosis. The published literature is reviewed and the possible pathogenesis of these nephropathies briefly discussed: either protein deposits, the nature of which has not been fully determined, or mesangial modifications induced by the filtered light chains.
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PMID:[Non-amyloid nephrotic syndrome: first isolated manifestation of a kappa light chain myeloma (author's transl)]. 12 16

We describe a 39-year-old man who developed kappa light chain nodular glomerulosclerosis with superimposed conspicuous crescent formation and extensive tubulointerstitial injury. The clinical picture was characterized by nephrotic syndrome and rapidly progressive glomerulonephritis. Incessantly progressive loss of renal function culminated in irreversible renal failure 7 weeks after initial manifestations of renal insufficiency. The patient has since been maintained on thrice weekly hemodialysis with chemotherapy for five years. At the time of pathologic diagnosis by renal biopsy, there was no evidence of multiple myeloma, and no serum M-component or Bence-Jones proteinuria was detected. An initial bone marrow aspirate revealed the presence of 0.6% atypical lymphocytes as the sole abnormality, although these were later identified as atypical plasma cells. These cells had also infiltrated the renal interstitium. Crescentic kappa light chain nodular glomerulosclerosis lacking evidence of plasma cell dyscrasia should be included in the differential diagnosis of rapidly progressive glomerulonephritis.
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PMID:Kappa light chain nodular glomerulosclerosis with conspicuous crescent formation and tubulointerstitial injury. Report of a case. 150 4

The clinical and necropsy findings on 7 patients with monoclonal immunoglobulin light chain deposition nephropathy are described (4 cases with a myelomatous and 3 with a non-myelomatous background). All patients had moderate proteinuria and progressive azotemia. Three myelomatous and all three non-myelomatous patients died from uremia after a mean time of 12 and 23 months, respectively, from the first presentation. Immunohistochemically, 6 patients had kappa, and one had lambda light chain deposition. Light microscopically, interstitial fibrosis, tubular atrophy and arteriolar hyalinosis were present in all cases. The glomeruli showed no changes (1 case), or displayed patterns of mesangial widening: mild (1 case), nodular (mesangial nodules, 4 cases) or global lobular expansion (1 case). Mesangial nodules were observed either with or without lamellation. Around the nodules, microaneurysms were seen in 2 cases. Mesangial nodular expansion was accompanied by crescents in 56% of the glomeruli in a male patient suffering from kappa light chain deposition nephropathy without myeloma. The present findings and a review of the literature indicate the following mesangial changes in light chain deposition nephropathy: 1. no changes, 2. mild expansion, 3. nodular expansion with and without lamellation, and 4. lobular expansion. Subtypes 2, 3 and 4 may be present in parallel, may occur with or without cellular proliferation, and may be accompanied by crescents. The term nodular glomerulosclerosis to describe mesangial nodular expansion is not completely correct, and hence its use is not recommended.
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PMID:Light chain deposition nephropathy in necropsy material. 176 89

A 60-year-old man with nephrotic syndrome and renal failure was found to have a IgD-lambda myeloma. Autopsy revealed advanced nodular glomerulopathy with codeposition of delta heavy and lambda light chains. Glomerular nodules were of two types: One (type I lesion) is caused by nodular expansion of the mesangium by deposition of abnormal immunoglobulin. The second type (type II lesion) is peripherally lamellated and is ascribed to repeated local mesangiolysis superimposed upon type I lesion. These type I and type II lesions correspond to the two types of nodules in diabetic glomerulosclerosis.
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PMID:Two types of glomerular lesion in non-amyloid immunoglobulin deposition disease: a case report of IgD-lambda myeloma. 211 26

The authors deal with the clinicopathology of the renal, alterations in light-chain disease in connection with 6 cases. The disease was recognized by the monotype (in 5 cases kappa, in 1 case lambda) immunoreactivity of the light-chain paraprotein deposited in the basal membranes of the renal tissue. Electron microscopic examinations proved the fine-granulated, electrodense character of the paraprotein. Multiple myeloma was found in 3 cases and plasma cell dyscrasia of non-tumorous characteristic in 3 cases in the background of the deposition. The renal involvement appeared clinically in the picture of proteinuria without nephrosis syndrome and in progressing azotemia. Chronic renal insufficiency developed during some months in 5 patients. Morphologically renal impairment manifested in interstitial fibrosis, tubular atrophy and ateriolar hyalinosis was seen. These were associated with different glomerular alterations, for instance in 3 cases with nodular glomerulosclerosis. In 1 patient with plasma cell dyscrasia of non-tumorous characteristic nodular glomerulosclerosis and semilunar formation was observed in 56% of the glomeruli. In an other patient with myeloma the simultaneous existence of cylinder nephropathy and light-chain nephropathy was demonstrated. Both observations are unusual phenomena in plasma cell dyscrasia.
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PMID:[Light-chain nephropathy]. 211 26

We report a case showing typical diabetic nodular glomerulosclerosis without retinopathy or other apparent clinical findings of DM except for impaired glucose tolerance. The 57-year-old man had a family history but no personal history of DM. In an extensive examination for DM, the results of funduscopy, daily profile of serum glucose and hemoglobin Alc were entirely within normal limits. However, the oral glucose tolerance test was abnormal. A renal biopsy showed typical nodular lesions (Kimmelstiel-Wilson's lesions). Previously, the interesting feature of transient proteinuria had been recognized. Although hypocellular nodular lesions by light microscopy are characteristic of diabetic nephropathy, renal amyloidosis, carbon disulfide intoxication, multiple myeloma and light chain disease, we concluded that the present lesions had resulted from diabetic nephropathy based on the family history, patient history, impaired glucose tolerance, immunofluorescent findings and electron microscopic observations.
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PMID:Nodular glomerulosclerosis in a patient showing impaired glucose tolerance. 225 Apr 5

The nodular lesion in renal glomeruli develops as a localized accentuation of mesangial volume augmentation. First described by Kimmelstiel and Wilson in 1936, it was soon accepted as the characteristic form of glomerular sclerosis in patients with long-term diabetes mellitus. It has for a long time been recognized that lesions very similar to nodular glomerulosclerosis may rarely occur in patients without diabetes. Clinical and pathological investigations of such cases have shown that glomerular nodules can also develop, 1. as a sequal to light chain deposit disease associated with plasma cell dyscrasia (e.g. myelomatosis), 2. in mesangioproliferative and membranoproliferative glomerulonephritis ("lobular" GN) and 3. in some cases of glomerular amyloid deposition. A survey is given of the characteristic light microscopy, ultrastructure and immunopathology of the glomerular nodules in these diseases. The differential diagnosis and pathogenesis is discussed.
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PMID:[Clinical pathology of the glomerulus--from phenomenon to entity. The nodular-lobular lesion]. 248 39

Two cases of renal impairment caused by the deposition of monoclonal immunoglobulin light-chain determinants in the glomerular mesangium and basement membranes as well as in the tubular basement membranes of the kidney are described (light-chain nephropathy). Both cases were associated with myeloma. Examinations by light microscopy, immunohistochemistry and electron microscopy are also described. Differential diagnoses on light microscopy include diabetic glomerulosclerosis (Kimmelstiel-Wilson lesions), amyloidosis and mesangiocapillary glomerulonephritis type 1.
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PMID:Light-chain nephropathy. A report of 2 cases. 249 64

A case of 66-years-old woman with mild renal failure due to deposition of K light chains on glomerular nodules, is reported. Monoclonal K light chains were found by immunofixation in serum and concentrated urine. Bone marrow examination showed a moderate increase of plasma cells, all stained for K light chains. Amyloid was not identified. No chemotherapy was started. Despite this, in the 15-months follow-up, renal function was preserved; no signs of myeloma, nor of extrarenal involvement were found. Careful follow-up is emphasized. The reason why light chains form amyloidoses or nodular deposits, like diabetic glomerulosclerosis of Kimmelstiel-Wilson, is briefly discussed, together with the differential diagnosis from other nephropathies.
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PMID:[Light-chain deposition disease. Presentation of a case]. 250 15

A 68-year-old female patient with multiple myeloma exhibited advanced nodular glomerulosclerosis. Immunofluorescence of the kidney showed kappa light chain deposition in the mesangium and in glomerular and tubular basement membrane. Isoelectric focusing and immunofixation of urinary proteins revealed an isolated kappa light chain with an unusually high isoelectric point of 8.4. Most light chain proteins have isoelectric points in the 4.6 to 6.7 range. Since loss of fixed negative charges may precede experimental glomerulosclerosis, it is proposed that this cationic circulating kappa chain may have interacted with glomerular polyanion, thereby inducing a nodular sclerotic reaction leading to irreversible renal damage.
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PMID:Nodular glomerulosclerosis associated with multiple myeloma. Role of light chain isoelectric point. 307 52


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