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)

Sixty-eight patients with plasmacytic neoplasia and osteosclerotic lesions were analyzed. Men predominated in this series. Mean age was 55.3 years and 26 patients were younger than 51 years at diagnosis. Early onset of disease was statistically different from multiple myeloma in general. Thirty patients had peripheral polyneuropathy and often neurological manifestations preceded other symptoms. Skeletal pain was less common, whereas hepatomegaly, splenomegaly, and lymphadenopathy were more common than in myeloma in general. Incidence of azotemia, hypercalcemia, high ESR, and anemia was lower than in myeloma. In one fourth of the patients, the number of skeletal lesions did not exceed three. Mean survival was less than 20 months from first symptom and 12 months from diagnosis. Mortality was related sometimes to polyneuropathy. Thus, in several aspects, plasmacytic neoplasia with osteosclerotic lesions is different from the classical multiple myeloma.
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PMID:Plasma cell neoplasia with osteosclerotic lesions. A study of five cases and a review of the literature. 22 10

From analysis of serum samples and clinical data from 632 patients with multiple myeloma it was determined that IgG was the commonest class of myeloma and that this type seemed to be the most benign. IgD myeloma and lambda light-chain disease were the most aggressive types; patients with these types were usually younger at diagnosis and more commonly had azotemia, osteolytic lesions and Bence Jones proteinemia. The sexes were equally represented in all but IgD myeloma, in which males predominated. Prognosis was more favourable when the M component had kappa light chains.
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PMID:Clinical manifestations of multiple myeloma: relation to class and type M component. 81 90

Physical and chemical characteristics of the abnormal immunoglobulin as well as the immunoglobulin type are important determinants of the clinical features and prognosis in myeloma. A review of 270 cases of myeloma studied by the Acute Leukemia Group B showed that survival was adversely affected by azotemia. The presence of Bence Jones proteinuria adversely influenced survival even in patients who were not initially azotemic. The presence of lambda-type Bence Jones proteinuria resulted in greatly shortened survival in contrast to kappa-type Bence Jones proteinuria in the absence, or presence, of a serum IgG M-protein. The adverse effect of lambda-type Bence Jones protein could not be demonstrated in patients with IgA myeloma. Patients with IgA myeloma were found to have a noticeably shorter survival than those with IgG myeloma.
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PMID:Correlation of abnormal immunoglobulin with clinical features of myeloma. 111 70

The effect of certain disease parameters on remission and survial time was evaluated in 482 patients with multiple myeloma treated with intermittent courses of melphalan-prednisone combinations. Increasing degrees of anemia, hypercalcemia, azotemia, and high serum myeloma protein levels were associated with progressive lifespan shortening. The short survival of patients with anemia and hypercalcemia was associated with short remissions in responding patients with these abnormalities. The extent of tumor mass was defined from specific laboratory parameters reported by Durie to be associated with large numbers of plasma cells. More advanced stages of myeloma were associated with higher frequencies and degrees of normal immunoglobulin depression. The response rate was not affected by the tumor mass grade, but increasing tumor mass was associated with a shorter lifespan. Greater degrees of tumor reduction were associated with longer remission and survival times. Patients in whom a marked tumor reduction was rapid had shorter survival and remission times than patients who responded more slowly.
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PMID:Prognostic factors in multiple myeloma. 117 23

A study was designed to evaluate the effectiveness of prednisone therapy in poor-risk patients with multiple myeloma. Patients were treated with melphalan alone or in combination with prednisone at doses of either 0.6 mg/kg or 0.3 mg/kg. The group of patients receiving melphalan and prednisone 0.6 mg/kg had significantly improved responses in hemoglobin, lowering of the M-protein concentration, and reduction of azotemia. Significant benefits attributable to prednisone were not demonstrated in the group receiving 0.3 mg/kg. Good responses have been shown to produce improved survival. The combination of melphalan and prednisone is effective in producing good responses, but the dose of steroids is important. A dose of prednisone of 0.6 mg/kg in gradually decreasing doses has been found to be effective in production of good responses, especially in the uremic patient.
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PMID:The use of low-dose prednisone and melphalan in the treatment of poor-risk patients with multiple myeloma. 123 29

We report a rare case of an immunoglobulin D (IgD) myeloma mass that occupied the lumbar epidural space. The patient presented with paraparesis combined with an extremely high concentration of serum IgD, and azotemia. A complete remission was obtained after combination chemotherapy, but the patient died from an epidural tumor in the high thoracic region 19 months after the onset of symptoms.
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PMID:Extraosseous epidural tumor of immunoglobulin D myeloma. 152 Sep 97

Serum hyperviscosity syndrome was diagnosed in 2 cats with multiple myeloma. Clinical signs included pale mucous membranes, dehydration, retinal hemorrhages, dilated and tortuous retinal vessels, seizures, head-tilt, nystagmus, systolic murmur, and gallop rhythm. Laboratory abnormalities included hyperglobulinemia, azotemia, hyperphosphatemia, nonregenerative anemia, and thrombocytopenia. Both cats had IgG monoclonal gammopathy, Bence Jones proteinuria, increased numbers of bone marrow plasma cells, and high values for relative serum viscosity. Renal disease was suspected in both cats. Cardiac hypertrophy was documented in 1 cat and was suspected in the other cat. Chemotherapy, using melphalan, prednisone, and vincristine, caused short-term remission in both cats, and plasmapheresis was used to lower serum protein concentration in 1 cat. Serum hyperviscosity syndrome rarely develops in cats, but should be suspected when monoclonal gammopathy exists with signs of neurologic, cardiac, or retinal disease.
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PMID:Serum hyperviscosity syndrome associated with multiple myeloma in two cats. 153 97

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

Monoclonal immunoglobulin deposition occurs in tissues as Congo Red binding fibrils in light chain amyloidosis, as less structured deposits in light chain deposition disease, and as similar but distinct deposits in light and heavy chain deposition disease. The nonamyloid forms were found in 13 patients who had evidence of plasmacytic dyscrasia by the immunohistochemical detection of immunoglobulin light chains of kappa or lambda class (with or without staining for a single heavy chain isotype) and by the absence of amyloid P component in tissue sections that did not show the birefringence characteristic of amyloid after Congo Red staining. All but two of the patients presented with proteinuria with or without azotemia. Clinical syndromes involving other organ systems were less common but occasionally severe. Four patients had overt multiple myeloma. Three others had hypercalcemia and mild bone marrow plasmacytosis but no lytic lesions. Analyses of immunoglobulin synthesis in bone marrow cells from seven patients showed excess light chains in all and incomplete light chains or heavy chain fragments in six, regardless of whether an intact monoclonal protein or related subunit was in the serum or urine. The fibrillar (amyloidotic) and nonfibrillar forms of monoclonal immunoglobulin deposition occur either in overt multiple myeloma or in the course of less neoplastically aggressive plasmacytic dyscrasias. Bone marrow cells from patients with either type produce immunoglobulin fragments that are related to those deposited in the affected tissues.
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PMID:Monoclonal immunoglobulin deposition disease: light chain and light and heavy chain deposition diseases and their relation to light chain amyloidosis. Clinical features, immunopathology, and molecular analysis. 210 17

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


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