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)

We describe a large experience with patients with end-stage renal disease (ESRD) whose primary cause was considered multiple myeloma, renal cell carcinoma and amyloidosis. Data were obtained by a collaboration of the majority of the ESRD Networks of the United States. The data presented reflect recent practice in the United States. Even though it is likely that some selection bias plays an important role, the demographic characteristics, choice of early treatment and overall survival data provide some assistance to physicians when advising their patients with these rare conditions. More detailed analyses are needed to assess the outcome by choice of treatment as well as the age- and treatment-adjusted survival for these diagnoses.
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PMID:Outcome of end-stage renal disease in patients with rare causes of renal failure. II. Renal or systemic neoplasms. 269 11

A prospective, 1-year study was performed to determine the causes of an ESR greater than or equal to 100 in patients admitted to a general medical ward in Harare, Zimbabwe. An ESR greater than or equal to 100 was found in 101 (12%) patients. Infection (46 patients) was the commonest cause, followed by malignancy (25), connective tissue disease (17), renal disease (8) and liver disease (5). The frequency of an ESR greater than or equal to 100 in these diagnostic groups was infection (28%), malignancy (44%), connective tissue disease (71%), renal disease (30%) and liver disease (24%). Pneumonia was the commonest infection diagnosed and the commonest cause of a markedly elevated ESR. Although myeloma was only the second commonest malignancy diagnosed it was the commonest malignancy causing an ESR greater than or equal to 100. In the largest group, infection, there was a significantly increased mortality in patients with an ESR greater than or equal to 100.
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PMID:Extreme elevation of the erythrocyte sedimentation rate in patients admitted to a general medical ward in Harare, Zimbabwe. 276 Sep 68

In connection with the analyses of 84 post-mortem examinations (47 men, 37 women, average age: 66.3 years) the author dealt with the renal complications of multiple myeloma. The signs of cylinder nephropathy, light-chain nephropathy, amyloidosis, nephrocalcinosis, urate nephropathy, acute renal insufficiency, renal vein thrombosis, acute and chronic pyelonephritis as well as the tumorous infiltration of the renal tissue have been sought for. The severity of the lesions were ranged into minimal, slight, moderate, and severe groups. On the basis of the semiquantitative morphological picture and the clinical data: 1. intact kidney (41 patients), 2. involvement of the kidney without azotemia (10 patients), 3. involvement of the kidney with azotemia (17 patients, serum creatinine level: greater than 177 mumol/l) and 4. renal involvement with chronic renal insufficiency associated with uremia (16 patients) were discerned. In the background of 33 cases (39%) with deteriorated renal function cylinder nephropathy was found most frequently (27 occasions) (32%). Every other complication occurred significantly less frequently e.g. amyloidosis or kappa-light-chain nephropathy occurred in 3 cases each.
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PMID:[Renal complications of multiple myeloma]. 279 87

We studied 29 patients affected by acute renal failure due to multiple myeloma with Bence-Jones proteinuria greater than 1 g/day to evaluate the effectiveness of plasma exchange in the treatment of severe myeloma nephropathy. Renal failure was severe enough to require dialysis in 24 cases, while the remaining 5 patients showed serum creatinine levels greater than 5 mg/dl. The patients were randomly allocated to Group I (15 patients undergoing plasma exchange together with corticosteroids, cytotoxic drug, hemodialysis only when needed) or to Group II (14 patients undergoing peritoneal dialysis together with corticosteroids and cytotoxic drug). In Group I Bence-Jones proteinuria decreased dramatically (P less than 0.01) with a significant increase in urine output (P less than 0.001), while Group II presented a slight reduction in Bence-Jones proteinuria without a significant increase in daily diuresis. Thirteen out the 15 Group I patients recovered renal function reaching serum creatinine levels less than or equal to 2.5 mg/dl in most cases. Only two patients in Group II improved renal failure well enough to stop dialysis. The one-year survival rate was significantly higher in Group I (66%) than in Group II (28%, P less than 0.01). We conclude that plasma exchange associated to chemotherapy rapidly removes large amounts of light chains, improves both renal function and long-term survival expectancies.
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PMID:Controlled plasma exchange trial in acute renal failure due to multiple myeloma. 304 77

This study evaluated the utility of the immunoperoxidase method as applied to bone marrow sections in the diagnosis of patients with monoclonal gammopathies. Intracellular immunoglobulin light chains were identified in fixed, decalcified bone marrow biopsy sections from 66 patients with monoclonal proteins, using an avidin-biotin-peroxidase complex immunoperoxidase method. In all cases the predominant light chain identified in the bone marrow biopsy correlated with the monoclonal light chain identified in the serum. In addition, a light chain ratio was defined that correlated with the clinical diagnoses. The light chain ratios were highest in patients with multiple myeloma and were significantly different from those with monoclonal gammopathy of undetermined significance. There was no correlation between level of serum monoclonal protein and light chain ratios. The ratios were also high in patients with macroglobulinemia, primary amyloidosis, and renal disease secondary to monoclonal proteins but without overt myeloma. Determination of light chain ratios differentiated patients with multiple myeloma from those with monoclonal gammopathy of undetermined significance and helped identify patients with end organ damage secondary to monoclonal proteins but without overt myeloma.
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PMID:Application of the immunoperoxidase technic to bone marrow trephine biopsies in the classification of patients with monoclonal gammopathies. 308 74

Two patients, one with myeloma (Patient 1) and the other with probable chronic lymphocytic leukemia (Patient 2), had reduced renal tubular phosphate reabsorption in the absence of hyperparathyroidism together with other features of the Fanconi syndrome, as consequences of the nephropathy associated with light-chain proteinuria. Both patients had hypophosphatemic osteomalacia, demonstrated for the first time in this condition by iliac bone histomorphometry after in vivo double tetracycline labeling, despite absence of bone pain or Looser zones. Neither patient was vitamin D-depleted, but plasma calcitriol level was normal in Patient 1 and low in Patient 2; only the latter patient had severe muscle weakness. Complete histologic correction of osteomalacia was achieved by treatment in accordance with the biochemical defects--oral phosphate therapy alone in Patient 1 and combined with calcitriol in Patient 2. Both patients are now symptom-free, five and three years after the initial diagnosis of bone disease and hematogenous malignancy. Thirteen previous instances of the same form of osteomalacia were reviewed; in most cases, the Fanconi syndrome developed before its probable cause became apparent. The Fanconi syndrome has also been reported in two cases of osteomalacia due to mesenchymal tumor, but not in osteomalacia associated with prostatic carcinoma. Light-chain nephropathy and consequent renal tubular dysfunction appears to be a third form of oncogenous osteomalacia.
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PMID:Hypophosphatemic osteomalacia and adult Fanconi syndrome due to light-chain nephropathy. Another form of oncogenous osteomalacia. 310 97

Acute renal failure developed in a patient with a normal serum creatinine level, after treatment with rifampin was begun for tuberculosis. Renal biopsy revealed an obstructive nephropathy due to tubular casts. Immunoperoxidase and immunofluorescence studies demonstrated the presence of heterogeneous light chains within these casts. This unique drug-induced renal disease is discussed with reference to the literature and to possible analogies with myeloma kidney.
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PMID:Light chain cast nephropathy and acute renal failure associated with rifampin therapy. Renal disease akin to myeloma kidney. 310 82

Excessive monoclonal light chain production and excretion may result in a variety of renal diseases which may collectively or individually be referred to as light chain nephropathy. Kappa light chains are more likely to produce tubular dysfunction and nodular nonamyloidotic glomerulosclerosis, while lambda light chains are more likely to be involved in the development of amyloidosis. The physicochemical reasons for this segregation are poorly understood. Affected patients may present with minor tubular dysfunctions, acute or chronic renal failure, mild proteinuria, or severe nephrotic syndrome. Underlying each is a dyscrasia of plasma cells or frank multiple myeloma with excessive production of monoclonal light chains. Electron microscopy and immunofluorescence studies of renal biopsies have been critical in defining these nephropathies and continue to be essential in establishing the diagnosis.
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PMID:Immunoglobulin light chain nephropathies. 312 Jan 37

To investigate the direct toxicity of human Bence Jones protein (BJP), individual nephrons of male Sprague-Dawley rats were perfused in vivo at 20 nl/min with an artificial tubule fluid (ATF) that contained no protein, a human kappa BJP (5 g/dl), or bovine serum albumin (5 g/dl), and proximal convoluted tubule function and morphology were examined. Perfusion with BJP perfusate for less than or equal to 5 minutes produced no changes (P = NS) in absorption of water, Jv, (1.09 +/- 0.20 vs. 1.50 +/- 0.25 nl/min/mm), chloride, JCl, (95 +/- 47 vs. 123 +/- 41 pEq/min/mm), and glucose, JG, (39 +/- 3 vs. 40 +/- 5 pmol/min/mm) compared to perfusions with only ATF. However, perfusion for at least 20 minutes with the same BJP perfusate produced decreased (P less than 0.025) in Jv (0.58 +/- 0.12 vs. 1.15 +/- 0.14 nl/min/mm) and JG (27 +/- 3 vs. 38 +/- 3 pmol/min/mm) compared to perfusions with ATF alone; the decrease in JCl (64 +/- 47 vs. 119 +/- 27 pEq/min/mm) did not reach statistical significance. Perfusion for 20 minutes with ATF containing albumin resulted in no changes in Jv (1.22 +/- 0.21 vs. 1.15 +/- 0.14 nl/min/mm), JCl (207 +/- 29 vs. 119 +/- 27 pEq/min/mm), and JG (31 +/- 1 vs. 38 +/- 3 pmol/min/mm), when compared to the ATF perfusions. Immunocytochemistry, immunofluorescence and immunoelectron microscopy of the BJP-perfused tubules demonstrated the kappa light-chain protein in endosomes and activated lysosomes. In addition, cellular desquamation and fragmentation, prominent cytoplasmic vacuolation, and focal loss of the microvillus border were found in the BJP-perfused tubules, but not in the albumin-perfused tubules. In conclusion, these functional and morphologic data show that a human kappa light-chain is toxic to the proximal convoluted tubule of the rat. This toxicity occurred in a time-dependent fashion when the lysosomal system was markedly activated. Direct damage of the tubule epithelium by BJP's may be involved in the development of the tubulointerstitial nephropathy associated with multiple myeloma.
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PMID:Human Bence Jones protein toxicity in rat proximal tubule epithelium in vivo. 312 60

Fifteen patients aged 31-74 years (five male, ten female) on renal biopsy showed intense linear deposits of light chains along tubular basement membranes (TBM) by immunofluorescence, and/or granular dense deposits on electronmicroscopy. Multiple myeloma was diagnosed in ten patients. The onset of myeloma and nephropathy was simultaneous in six patients; nephropathy preceded or followed the diagnosis of myeloma in three and one patients respectively. The mode of onset of nephropathy was acute or rapidly progressive renal failure in five cases, chronic renal failure in seven, and heavy proteinuria in three. Only two patients had normal renal function at biopsy. Serum monoclonal component was kappa in five patients, IgG kappa in three, IgD kappa in one, IgG lambda in one, IgA lambda in one, absent in three, and not detected in one. On light microscopy eight cases had nodular glomerulosclerosis, three cast nephropathy and 14 TBM thickening. Immunofluorescence for monoclonal light chain(s) was positive in 11 of 13 cases. Electron microscopy showed finely granular deposits in the inner side of glomerular basement membranes (GBM) and the outer side of TBM in 11 of 11 tested cases. The evolution was towards chronic renal failure in 12 patients (six of whom required dialysis), death in two, unknown in one. Four patients died after a period of dialysis, from infections or cardiovascular complications.
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PMID:Light chain nephropathy: histological and clinical aspects in 15 cases. 314 81


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