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)

An 82 year old woman with suspected Bence Jones myeloma developed intractable fluid retention presumed secondary to cardiac failure. In addition she experienced angina pectoris, and required permanent cardiac pacing for symptomatic sinus bradycardia. Postmortem studies revealed prominent myocardial and renal deposits of lambda light-chains which were Congo Red negative, and had a non-fibrillar ultrastructure. Non-amyloidotic light-chain deposition is uncommon, and a rare cause of cardiac disease. Previous work regarding possible pathogenetic mechanisms, clinical and laboratory features and treatment is reviewed.
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PMID:Systemic lambda light-chain deposition presenting with predominant cardiac involvement. 841 52

Along with an introductory summary concerning practically with all types of amyloidoses the authors report on a case of a primary amyloid tumor in a female patient, which resulted the eight after a computer-assisted literature search. Amyloid deposits in the breast represent an extremely rare event, which has been described in three clinical settings, in patients with reactive or secondary amyloidosis, in patients with immunocytic amyloidosis (this term including both the so-called primary form and the myeloma-associated form), and finally even in patients who are well and otherwise asymptomatic. Amyloid deposits in the breast can occur in the course of a systemic involvement as well as in form of a localized or organ-limited disease ("amyloid tumor"). Further the latter form is qualified as a "secondary amyloid tumor" (in those patients affected by certain neoplastic diseases, plasmacellular or non plasmacellular, or by a chronic infectious-inflammatory-dysreactive process) or as a " primary amyloid tumor" in those who are found free of any disease and of any other amyloid deposits. The case the authors report on deals with a lady who was admitted due to a breast lump which mammographically was thought suspicious for malignancy by virtue of a cluster of variously sized microcalcifications. At histology the lesion was diagnosed as an amyloid deposit on special stains and disclosed of the AL type with Congo red stain on sections previously treated with KMn04, according to standard methods.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Primary amyloid tumor of the breast. Case report and review of the literature]. 853 10

AA-amyloid has been produced experimentally in animal models, allowing the study of mechanisms involved in AA-amyloidogenesis, but those involved in renal AL-amyloidogenesis have not been adequately investigated due, in part, to lack of appropriate in vitro models. Rat and human mesangial cells were grown on a human extracellular matrix (Amgel) derived from normal tissues and on coverslips in the presence of 10 microliters of amyloid enhancing factor (AEF) per milliliter of media and 10 micrograms/ml monoclonal lambda light chains (LCs) obtained from two patients with AL-amyloidosis. Two additional lambda LCs derived from the urine of patients with myeloma and tubulointerstitial renal disease were used as controls. To verify amyloid deposition, light and electron microscopic examination, as well as Congo red and thioflavin T staining, were performed on samples incubated under different experimental conditions. Intracellular and extracellular amyloid was identified in samples incubated for 24 hours with human mesangial cells (for 48 hours with rat mesangial cells), amyloidogenic monoclonal LCs, and AEF. The amount of amyloid detected, which increased with longer incubation times, was found to be most abundant at 14 days. Amyloid was not present in cultures of mesangial cells incubated with amyloidogenic LCs alone or in the absence of mesangial cells. Likewise, incubation of mesangial cells with amyloidogenic LC or AEF separately or amyloidogenic LC in the presence of AEF but without mesangial cells did not result in amyloid formation. Amyloid was not seen when LCs obtained from the urine of patients with tubulointerstitial renal disease were incubated with AEF and mesangial cells. AL-amyloid production requires all three components--mesangial cells, amyloidogenic LCs, and AEF. In addition, amyloid was detected intracellular in mesangial cells, supporting the hypothesis that the production of AL-amyloid in the kidney requires intracellular processing by these cells. This system provides a unique experimental model to study renal AL-amyloidogenesis and a platform to explore mesangial cell-matrix interactions.
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PMID:In vitro AL-amyloid formation by rat and human mesangial cells. 856 93

A 77-year-old man presented with jaw claudication, arthralgias and myalgias, weight loss, marked fatigue, and thickened temporal arteries. No vasculitis was seen on the temporal artery biopsy specimen, but amyloidosis was suspected and confirmed with Congo red staining. Subsequent bone marrow biopsy revealed multiple myeloma. Although the patient initially was thought to have temporal arteritis, the results of temporal artery biopsy directed further investigations that led to the diagnosis of systemic amyloidosis. Systemic amyloidosis should be considered in the differential diagnosis when patients, especially men, present with clinical findings suggestive of temporal arteritis but without evidence of vasculitis in temporal artery biopsy specimens.
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PMID:Systemic amyloidosis with temporal artery involvement mimicking temporal arteritis. 910 75

To assess the value of fine-needle aspiration (FNA) cytology for the diagnosis of amyloid, we retrospectively studied all FNA cases diagnosed as having amyloid during a 6-yr period (1990-1996). FNA was performed on both superficial and deep locations. A total of 6 cases containing amyloid was studied, including primary medullary thyroid carcinoma, metastatic medullary thyroid carcinoma to a vertebrae, multiple myeloma, squamous-cell carcinoma of the lung metastatic to a hilar lymph node, primary pulmonary amyloid, and amyloid tumor in a vertebral body in a patient with primary systemic amyloidosis. Despite the location or disease association, the cytologic appearance of amyloid in all cases was similar. On Diff-Quik stain, amyloid appeared as amorphous, irregular, waxy basophilic to metachromatic clumps of material. Papanicolaou stain revealed cyanophilic to organophilic clumps of material with occasional prominent fissures. In all 6 cases, amyloid was confirmed by Congo red stain and in 3 cases by a thioflavin T stain. In 4 of the 6 cases (67%), amyloid was associated with an underlying malignancy. In 3 cases malignant cells were admixed with the amyloid, and in another case malignancy was present at a distant site. We conclude that FNA biopsy is a helpful initial procedure for the evaluation of patients with amyloid deposits. The clinical implications of amyloid found in any particular body site include both benign and malignant conditions. The presence of an associated neoplasm must be especially considered in the differential diagnosis of amyloid deposits.
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PMID:Fine-needle aspiration cytology of amyloid associated with nonneoplastic and malignant lesions. 955 61

Amyloid deposition in skeletal muscle is a well-recognized but rare occurrence. Sixteen such cases seen in a 17-year period (1979 to 1996) out of a total of 3,937 muscle biopsy specimens (0.004%) form this study group. Either Congo red or sulfated alcian blue stains were routinely performed in each biopsy to screen for amyloid. Patients in this study (eight men, eight women) ranged in age from 42 to 90 years (mean, 61 years) at initial presentation. The most common symptoms at presentation included weakness/fatigue (n = 10), autonomic symptoms (n = 8), and weight loss/decreased appetite (n = 7). Five patients had a concomitant malignancy (myeloma, n = 3; malignant carcinoid tumor, n = 1; melanoma, n = 1). Two patients had known hereditary forms of amyloidosis. Five patients had amyloid diagnosed on another organ biopsy (excluding peripheral nerve). Histologically, amyloid was deposited in the interstitium or perivascular region in 14 muscles and endomysial region in seven muscles. All cases were confirmed with Congo red staining (apple green birefringence) or by electron microscopic identification of fibrillary amyloid material. Scattered angular atrophic esterase-positive muscle myofibers indicative of acute denervation atrophy were seen in 14 muscles. Eight muscles showed small group atrophy, and seven showed myofiber type grouping. Scattered regenerating muscle fibers were seen in nine cases, degenerating myofibers in six, and foci of chronic endomysial and perivascular inflammation in two. Four muscles showed type II muscle fiber atrophy. A concomitant sural nerve biopsy specimen was evaluated in seven patients; all seven contained amyloid, confirmed either by Congo red staining or electron microscopic examination. In two nerves, there was a mild loss of myelinated axons; four had a moderate loss, and one, severe loss. Six of seven nerves showed predominantly axonopathic changes. In conclusion, (1) the prevalence rate of amyloid myopathy in muscle biopsy specimens was low (in this series, 0.004%); (2) only a minority of patients had multiple myeloma, and most presented with muscle weakness/fatigue or autonomic symptoms; (3) most of the muscles showed neurogenic features histologically; (4) all concomitant sural nerve biopsy specimens contained amyloid, and most showed a predominance of axonopathic changes.
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PMID:Amyloid myopathy: clinicopathologic study of 16 cases. 959 69

A 57-year-old man with monoclonal gamma-globulinemia was admitted because of edema and proteinuria. A renal biopsy specimen showed lobular glomerulonephritis associated with deposition of material that was positive for IgG, C3, C1q, fibrin, kappa light chain, and lambda light chain but was not stained by Congo red. Glomeruli showed massive electron-dense deposits with two kinds of unusual, highly organized crystalline structures in the mesangial matrix and peripheral capillary loops. Clinically, the patient had nephrotic syndrome, microscopic hematuria, and hypertension. No Bence-Jones protein or cryoglobulin was found in the urine or serum. Immunoelectrophoresis of blood and urine revealed increased IgG-lambda paraprotein, but no free light chains were found. This case was not associated with amyloidosis, systemic lupus erythematosus, light chain deposition disease, cryoglobulinemia, or multiple myeloma. Immunotactoid glomerulopathy was diagnosed. Treatment with oral prednisone was effective for the management of nephrotic syndrome and renal dysfunction. Glomerular deposition of two kinds of microtubular structure in immunotactoid glomerulopathy has rarely been reported.
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PMID:A case of immunotactoid glomerulopathy with unusual microtubular deposits. 961 98

We herein report on the first two primary sequences (BOU and RAC) of monoclonal light chains of the lambda type responsible for nonamyloid lambda light chain deposition disease. Both patients were affected with severe forms of myeloma complicated with renal failure. The pathological presentation typically featured Congo red-negative deposits along tubular basement membranes but differed somewhat from the typical "Randall-type" kappa light chain deposition disease: they lacked the prominent glomerulosclerosis pattern often featuring nonamyloid kappa deposits and were associated with cylinders or myeloma casts. Both protein sequences were deduced from those of the corresponding complementary DNAs in the bone marrow plasma cells. For each chain, products of three independent amplifications by polymerase chain reaction were sequenced and found to be identical. BOU and RAC lambda mRNAs had a normal overall structure consisting of Vlambda2 segments rearranged to Jlambda2Clambda2 but displayed a number of unusual features within their primary sequences. These substitutions are likely responsible for changes in light chain conformation that promote their aggregation and deposition along renal tubule basement membranes.
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PMID:Complete primary sequences of two lambda immunoglobulin light chains in myelomas with nonamyloid (Randall-type) light chain deposition disease. 966 93

A case of primary localized amyloid tumor of the breast is described. It is an extremely rare condition and has not been seen in literature in Japan. A 76-year-old woman visited a hospital because of a painless, hard mass of the right breast. A relatively well demarcated, calcified mass was excised under clinical diagnosis of fibroadenoma. Histologically, massive eosinophilic amorphous material was deposited in breast stroma. It was stained red-orange by Congo red and displayed apple-green birefringence under polarized light. The staining persisted after incubation with KMnO4 and immunolabeling by immunoglobulin chi-light chain antiserum, consistent with the amyloid of AL (Achi) type. Osseous metaplasia with bone marrow cavity, foreign body type giant cells in response to amyloid, and scattered plasma cell infiltration were also recognized. Osseous metaplasia in the breast amyloid tumor has been reported in only one case before. To date, the patient has not developed any clinical or laboratory evidence of systemic amyloidosis or multiple myeloma.
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PMID:Primary localized amyloid tumor of the breast with osseous metaplasia. 970 Oct 18

We report a case of light chain deposition disease associated with multiple myeloma revealed by congestive heart failure. Endomyocardial biopsy showed Congo red negative deposits and lambda light chain deposits by the immunofluorescence method. After chemotherapy with melphalan and prednisone, complete remission of multiple myeloma and major improvement in cardiac condition were observed.
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PMID:Congestive heart failure: revealing light chain deposition disease. 1074 55


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