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)

Vaccine-induced levels of antibody to Streptococcus pneumoniae of approximately 250-300 ng of antibody nitrogen/ml are protective against pneumococcal disease. Side effects of vaccination are not severe and are generally confined to local reactions at the site of inoculation. Patients with a documented high risk of acquiring pneumococcal disease include the elderly, especially those with underlying cardiopulmonary disease, and those with sickle cell anemia, Hodgkin's disease, a renal transplant, multiple myeloma, asplenia, and nephrotic syndrome. People with insulin-dependent diabetes mellitus or renal failure do not appear to be at high risk. All of these groups, except those with multiple myeloma, respond to vaccine with levels of antibody that are protective for many but not all of the serotypes included in the vaccine. Immunosuppression, splenectomy, and hemoglobinopathy depress antibody response. Duration of vaccine-induced antibody is unknown but may be shorter than that in normal persons. Preliminary guidelines for vaccination are proposed.
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PMID:Assessment of the antibody response to pneumococcal vaccine in high-risk populations. 702 58

Three cortisol fractions, protein-unbound (U-F), transcortin-bound (Tr-F) and albumin-bound cortisol (Al-F) were measured in patients with dysproteinemia by a newly devised isocolloidosmolar equilibrium dialysis method. Total cortisol (Total-F) concentrations in patients with liver cirrhosis (LC), anorexia nervosa (AN) and cachexia due to cancer (CA) were higher than in normal subjects, and those in patients with nephrotic syndrome (NS) and multiple myeloma (MM) remained within the normal range. In all groups of patients, the U-F concentration, which is believed to be the sole active fraction of cortisol, showed significantly higher values than in the normal subjects. We, therefore attempted to find which of the two binding proteins contributes to the elevated U-F concentrations. Concentrations of each cortisol fraction are greatly changed by alterations in the Total-F concentration. We therefore compared the Tr-F against Total-F and Al-F, and U-F against Total-E of patients with those of normal subjects. It was found that decreased transcortin-binding and not albumin-binding in the patients with cirrhosis, nephrotic syndrome and myeloma contributed to an increase in the U-F concentration. Although decreased binding of albumin due to hypoalbuminemia was found in LC, NS, MM, CA and AN, it had relatively little effect on cortisol distribution in the serum.
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PMID:The serum concentrations of unbound, transcortin bound and albumin bound cortisol in patients with dysproteinemia. 718 82

The appearance of amyloidosis during the course of multiple myeloma is a well known fact and has an overall incidence of 6 to 15%. However, the total transformation of a plasmocytoma into a voluminous amyloid tumor is a very rare event. A female patient was diagnosed of lambda light chain disease after developing a conspicuous rib plasmocytoma over the same region where a pathological fracture had appeared three years before. She was treated with discontinuous courses of melphalan and methyl-prednisolone, and developed a reversible nephrotic syndrome and a pathological fracture of the right clavicle. At necropsy there was generalized amyloidosis and complete substitution of the rib plasmocytoma by amyloid substance, with another important accumulation of amyloid in the region of the clavicular fracture. The present concepts on amyloidogenesis in multiple myeloma are reviewed, and the peculiarities of the present case together with the possible role of initiating factors and the effects of therapy are discussed. The case herein reported appears to represent a human model of focal amyloidogenesis in myeloma.
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PMID:[Transformation of a rib plasmocytoma into an amyloid tumor (author's transl)]. 725 34

The renal concern in a multiple myeloma (MM) case has a frequency of 50% and causes a worsening of the disease with a survival average of about 12 months. The monoclonal light free chains (CLL) produced in excess by the plasmacytes are present in the urine as proteinuria of Bence Jones (PBJ) in 60-70% of patients affected by MM. They represent the major pathogenetic factor of the nephropathy in course of MM as they can deposit in shape of intratubular "casts" in the myeloma casts nephropathy (MCN). In some worse cases, dehydration or hypercalcaemia can cause an irreversible acute renal insufficiency (RI). It is therefore important in a patient affected with MM with PBJ to prevent, locate and opportunely treat these situations which worsen the nephropathy. Beside the tubular cast nephropathy, the CLL "accumulate" in the kidney even though with a lower frequency compared to MCN, in the light chains deposition disease (LCDD) and in the amyloidosis AL (AL). LCDD is characterized by a deposit of nodular amorphous materials PAS positive in the glomerulus and sometimes even in the tubulus. It usually presents itself as a chronic RI and a proteinuria causing nephrotic syndrome (NS). This quickly evolves into uraemia and its evolution can be lessened by the MM treatment. AL in course of MM also reveals with a chronic RI and NS. CLLs deposit in the typical fibrillar structure, on the vessel walls, in the glomerulus, in the mesangium and can be marked out with the Congo red colouring and the subsequent green birefringence through microscope with polarized light. Prognosis of AL is extremely severe and no benefit is given by the treatment of the hematological illness. It is therefore absolutely necessary to study the renal histology through biopsy when MM is grade B, that is, with serumal creatinine above 2 mg/dl as: MCN imposes the MM treatment programme in order to reduce the tubular excess of PBJ and to attempt to make RI reversible; MCN with tubular atrophy and interstitial fibrosis results in an unfavourable prognosis as it expresses a nephropathic irreversibility due to the loss nephrons. It will therefore necessary to start on a renal substitutional treatment programme. Renal damage in course of MM is not always tubular, rather an unexpected glomerular damage of LCDD or amyloidosis AL type can be found.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:[The kidney in multiple myeloma. The physiopathological and clinical aspects]. 818 90

Renal insufficiency, which is present initially in almost half of patients with multiple myeloma, usually results from myeloma kidney or hypercalcemia. Neither the class of light chain nor the isoelectric point plays an important role in kidney failure. Acute renal failure must be treated with appropriate fluids and with electrolytes and hemodialysis if necessary. Plasma exchange may be helpful, but has not been proven as such. The presence of a nephrotic syndrome and a monoclonal kappa or lambda light chain in the urine almost always indicates primary amyloidosis (AL) or light-chain deposition disease. Amyloid fibrils must be distinguished from the fibrils of immunotactoid glomerulopathy.
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PMID:Monoclonal proteins and renal disease. 819 2

We report a case of a 40-year-old woman with benign monoclonal gammopathy who developed focal segmental glomerulosclerosis associated with widespread deposition of large crystalline inclusions in the glomerular podocytes. Electron microscopy showed that the crystalline structures were electron dense and needle shaped or polygonal. At a higher magnification, they were seen to be surrounded by unit membranes and to feature a longitudinal filamentous substructure. These inclusions showed positive immunohistochemical staining for kappa light chain. Clinically, the patient had shown nephrotic syndrome on the first medical examination, and later revealed chronic renal failure and monoclonal gammopathy of the immunoglobulin G kappa type, but no evidence of multiple myeloma or other lymphoproliferative diseases. This patient seems to have had a rare form of glomerular involvement associated with benign monoclonal gammopathy.
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PMID:Crystalline inclusions in the glomerular podocytes in a patient with benign monoclonal gammopathy and focal segmental glomerulosclerosis. 820 69

The 9697 electrophoretograms performed over an 8 year period were reviewed to identify the frequency and clinical associations of the finding of a prominent transthyretin band in serum or urine, the concentration of which was equal to or greater than a 64 mg/dL protein calibrator. All samples were electrophoresed at a constant 90 V using agarose gels with a barbital buffer pH 8.6 and Ponceau S staining. Reference calibrators were used as standards to identify increased transthyretin bands and the patients' clinical records were subsequently reviewed. High values were found in 46 patients' sera and a further nine patients' urines representing 0.57% of the total workload. Renal impairment was present in 58% of cases including those with chronic renal failure, the nephrotic syndrome and paraproteinaemia. The high levels were not persistent in three myeloma cases where there was a recovery in renal function following chemotherapy. In some nephrotics, a high urine transthyretin may be secondary to a general hepatic albumin and transport protein synthesis response to severe proteinuria. Why the serum transthyretin was elevated in many other cases remains unclear.
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PMID:Clinical associations of an increased transthyretin band in routine serum and urine protein electrophoresis. 830 23

Investigation of recurrent venous thromboembolic events in a 46-year-old man with progressive IgG kappa (total serum IgG, 74.3 mg/ml) multiple myeloma revealed profound reductions in free protein S (PS) antigen (<0.l U/ml) and PS activity (0.33 U/ml). Total PS antigen, protein C, antithrombin III, and C4b-binding protein levels were within normal limits. The patient had no family history suggestive of a congenital PS deficiency and no history of thrombosis predating the diagnosis of his plasma cell dyscrasia. Patient IgG was isolated from serum using a protein A-sepharose affinity column and characterized. PS-dependent clotting assays (Staclot Protein S, Diagnostica Stago, Asnieres sur-Seine, France) performed on normal pooled plasma mixed with dilutions of patient IgG (0.0-33.0 mg/ml) revealed a dose-dependent neutralization of PS activity by 43%. Total and free PS antigen levels were measured using Laurell rocket electroimmunodiffusion (Assera-Plate Protein S, Diagnostica Stago), which revealed a similar dose-dependent reduction in free PS antigen but preserved normal total PS antigen. Free PS antigen was reduced by 77% to 0.23 U/ml using an IgG concentration (16.5 mg/ml) less than one-fourth of that of the patient at time of serum collection. Specific binding of the patient IgG to commercially available purified human PS was demonstrated by Western immunoblot analysis. Whereas acquired free PS deficiency has been previously reported in association with nephrotic syndrome, inflammatory bowel disease, HIV infection, and varicella infection, this is the first reported case of a hypercoagulable syndrome associated with acquired free PS deficiency and multiple myeloma.
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PMID:Acquired free protein S deficiency associated with multiple myeloma: a case report. 860 34

We present the case of a 57-year-old woman with a thrombus in the right renal vein and the inferior vena cava that disappeared spontaneously during 6 months of observation. She had no thrombus-related disease such as kidney cancer, dehydration, multiple myeloma, nephrotic syndrome, or abnormal coagulability. While various examinations were being performed over a 2 month period the thrombus regressed spontaneously. After 6 months of follow-up the thrombus could not be seen on abdominal computerized tomography scan. Twenty months after disappearance of the thrombus the patient is doing well and has no recurrence of thrombus.
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PMID:Idiopathic and spontaneously regressing thrombus in right renal vein and inferior vena cava. 917 73

Data compiled during the 1970s and early 1980s indicated that during these periods, membranous nephropathy was the most common cause of unexplained nephrotic syndrome in adults, followed in order of frequency by minimal-change nephropathy and focal segmental glomerulosclerosis (FSGS). However, we and others recently reported an increase in the incidence of FSGS over the past two decades, and the number of cases of FSGS diagnosed by renal biopsies in these centers now exceeds the number of cases of membranous nephropathy. Nonetheless, as a substantial fraction of patients with FSGS do not have the nephrotic syndrome, it remained unclear as to what extent the relative frequencies of FSGS and other glomerulopathies as causes of the nephrotic syndrome have changed over this time. To address this concern, we reviewed data from 1,000 adult native kidney biopsies performed between January 1976 and April 1979 and from 1,000 biopsies performed between January 1995 and January 1997, identified all cases with a full-blown nephrotic syndrome of unknown etiology at the time of biopsy, and compared the relative frequencies with which specific diseases were diagnosed in these latter cases between the two time intervals. The main findings of this study were that, first, during the 1976 to 1979 period, the relative frequencies of membranous (36%) and minimal-change (23%) nephropathies and of FSGS (15%) as causes of unexplained nephrotic syndrome were similar to those observed in previous studies during the 1970s and early 1980s. In contrast, from 1995 to 1997, FSGS was the most common cause of this syndrome, accounting for 35% of cases compared with 33% for membranous nephropathy. Second, during the 1995 to 1997 period, FSGS accounted for more than 50% of cases of unexplained nephrotic syndrome in black adults and for 67% of such cases in black adults younger than 45 years. Third, although the relative frequency of nephrotic syndrome due to FSGS was two to three times higher in black than in white patients during both study periods, the frequency of FSGS increased similarly among both racial groups from the earlier to the later period. Fourth, the frequency of minimal-change nephrotic syndrome decreased from the earlier to the later study period in both black and white adults. Fifth, the relative frequency of membranoproliferative glomerulonephritis as a cause of the nephrotic syndrome declined from the 1976 to 1979 period to the 1995 to 1997 period, whereas that of immunoglobulin A nephropathy appeared to increase; the latter accounted for 14% of cases of unexplained nephrotic syndrome in white adults during the latter study period. Finally, 10% of nephrotic adults older than 44 years had AL amyloid nephropathy; none of these patients had multiple myeloma or a known paraprotein at the time of renal biopsy.
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PMID:Changing etiologies of unexplained adult nephrotic syndrome: a comparison of renal biopsy findings from 1976-1979 and 1995-1997. 937 Jan 76


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