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 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

Humoral immunity involves molecules in solution in biological fluids including effectors of non specific immunity (e.g. complement, cytokines) and specific immunity (antibodies) as well. Acquired humoral immunodeficiences are often multifactorial in origin and associated with defects of cell-mediated immunity. The most common etiologies are those of iatrogenic immunodeficiencies: surgery (especially splenectomy), radiotherapy, chemotherapy of leukemia and cancer, immunosuppressive treatments in organ transplanted patients. Protein-caloric malnutrition also induces cellular and humoral immunodeficiencies. Among other causes, three types of diseases may induce defective antibody production: 1/B cell neoplasias (e.g. multiple myeloma, chronic lymphocytic leukemia...) 2/renal diseases (nephrotic syndrome, renal insufficiency) and 3/various infectious diseases, including AIDS. Some principles of prevention and treatment of secondary humoral immunodeficiencies are given.
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PMID:[Secondary deficiencies of humoral immunity]. 204 15

A 59-year-old woman with nephrotic syndrome was diagnosed as having primary amyloidosis based on the detection of amyloid deposition (AL-protein) in the esophagus and kidneys. Bone marrow aspirate showed plasmocytic proliferation, leading to a diagnosis of multiple myeloma (IgG lambda-type). In addition, a very rare translocation t(1; 20) (q21; q11) was seen by chromosomal analysis of both the bone marrow and peripheral blood.
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PMID:Multiple myeloma associated with amyloidosis and t(1;20)(q21;q11) translocation. 210 67

A 43-year-old man was admitted to our hospital because of legs edema and periorbital edema in Dec. 1983. Laboratory findings showed massive proteinuria (3.7 g/day), Bence Jones protein (BJP) in urine, and hypoproteinemia. Peripheral blood examinations were normal and a bone marrow aspiration showed hypocellularity with slight increase of monocytes and plasma cells. Serum immunoelectrophoresis showed two M-components (IgG kappa, IgA lambda). Serum IgG was 1,690 mg/dl, IgA 379 mg/dl and IgM 160 mg/dl. No remarkable findings were obtained in bone survey, Ga-scintigraphy and rectal biopsy, and a diagnosis of diclonal gammopathy with nephrotic syndrome was made. In Aug. 1986, serum IgA started to increase rapidly with concomitant decrease IgG. He died of pneumonia due to pancytopenia in Dec. 1986, when serum IgG was 450 mg/dl, IgA 1,014 mg/dl, and IgM less than 39 mg/dl. Immunoelectrophoresis showed two M-components (IgG kappa, IgA lambda) in serum and BEP (kappa, lambda), IgG (kappa) and IgA (lambda) in urine. An autopsy showed massive infiltration of myeloma cells which were positive for lambda light chain in bone marrow, suggesting a development of myeloma from a diclonal gammopathy in about 3 years.
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PMID:[Diclonal gammopathy (IgG kappa, IgA lambda) with nephrotic syndrome terminating into IgA lambda myeloma after three years--report of a case]. 210 4

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

Proteinuria is frequently found in multiple myeloma and related disorders. Immunofixation electrophoresis is very helpful for the identification and characterization of the monoclonal component. In multiple myeloma, the presence of Bence-Jones (BJ) proteinuria is significantly associated with renal failure. The coexistence of BJ proteinuria and the nephrotic syndrome in myeloma patients should suggest AL amyloidosis or light chain deposition disease. In the latter, BJ proteinuria is absent in 30% of the cases and diagnosis is based on the demonstration of the monoclonal light chain deposits in tissues, predominantly in kidneys.
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PMID:Proteinuria in multiple myeloma and related diseases. 212 83

We report a 49-year-old woman who developed lobular glomerulonephritis with prevalent deposition of material positive for IgG, C1q and lambda light chain, but which was not stained by Congo red. Glomeruli revealed massive electron-dense deposits with a microlamellar structure in the mesangial matrix and peripheral capillary loops. Clinically, the patient had nephrotic syndrome, microhematuria and hypertension. No Bence-Jones protein or cryoglobulin was found in the urine or serum. Anti-DNA antibody was positive, but systemic lupus erythematosus (SLE) was ruled out by repeated serological examinations. Immunoelectrophoresis of blood and urine revealed increased IgG-lambda paraprotein, but no free light chains were found. We reviewed 54 cases reported in the literature, which showed organized crystalline structures on ultrastructural examination, but were unassociated with amyloidosis, SLE, cryoglobulinemia or multiple myeloma. The present patient is the first reported to have exhibited a combination of glomerulonephritis with organized deposits, monoclonal IgG lambda paraproteinemia, and the presence of anti-DNA antibody.
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PMID:Microlamellar structures in lobular glomerulonephritis associated with monoclonal IgG lambda paraproteinemia. A case report and review of the literature. 212 87

The major causes of renal insufficiency, present initially in half of the patients with multiple myeloma, are "myeloma kidney" and hypercalcemia. There is no reliable evidence that the isoelectric point (pI) or the type of light chain has an important role in renal failure. Acute renal failure must be treated vigorously with appropriate fluids and electrolytes as well as hemodialysis if necessary. The benefit of plasma exchange has not been proven. Primary systemic amyloidosis or light-chain deposition disease is the usual cause of the nephrotic syndrome in patients with monoclonal gammopathy. The monoclonal gammopathies are a group of disorders that are characterized by the proliferation of plasma cells producing a homogeneous, monoclonal protein (M-protein).
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PMID:Monoclonal gammopathies and the kidney. 249 46

A 57-year-old woman who had had renal amyloidosis with nephrotic syndrome for five years was found to have the Fanconi syndrome and monoclonal lambda light-chain proteinuria. The amyloidosis of this patient was of primary type on the basis of the permanganate-resistant amyloid, the presence of monoclonal light-chain proteinuria, and the absence of clinical and histologic evidence of multiple myeloma. To best of our knowledge, this is the first case report in which primary amyloidosis with monoclonal lambda light-chain proteinuria was accompanied by adult Fanconi syndrome.
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PMID:Adult Fanconi syndrome in primary amyloidosis with lambda light-chain proteinuria. 250 72

Amyloidosis may present with involvement of a variety of organ systems. Cutaneous involvement is a relatively common finding in patients with systemic amyloidosis. The occurrence of bullous skin lesions, however, is rare; only a few such cases have been previously reported. We describe a patient who presented with a subepidermal bullous skin disease initially thought to be bullous pemphigoid based on both clinical and histologic appearances. The patient subsequently developed the nephrotic syndrome. Biopsy specimens of the skin and kidney showed involvement of both organs with amyloid, and amyloid was later found in the spleen, heart, and nervous system. No subsequent evidence of myeloma was found in this patient. The clinical, histopathologic, immunofluorescent, and electron microscopic findings of systemic amyloidosis are discussed.
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PMID:Bullous amyloidosis. 265 87


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