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)

Marrow transplantation is effective treatment for a number of hematological diseases in patients under the age of 50 who have an HLA-identical sibling donor. It is successful in the treatment of aplastic anemia with 70-85% long-term survival. It offers 10-30% apparent cures for patients with acute leukemia who have relapsed at least once, and for those with chronic myelocytic leukemia in blast crisis. Although still somewhat controversial, it appears to be the treatment of choice for patients with acute nonlymphoblastic leukemia in first chemotherapy induced remission, and for those with chronic myelogenous leukemia in the chronic phase since approximately 50-60% of these patients experience long-term, disease-free survival. Patients with acute lymphoblastic leukemia grafted in second or subsequent remission may expect a 30% "cure" of their disease. Marrow grafting is the only effective treatment for many patients with inherited immunologic deficiencies and certain genetic storage diseases. Cures of congenital Fanconi's anemia, Blackfan-Diamond anemia, osteopetrosis, paroxysmal nocturnal hemoglobinuria and thalassemia major have been achieved. Marrow transplantation is being explored for the therapy of patients with lymphoma, Hodgkin's disease, preleukemia, multiple myeloma, hairy cell leukemia, small cell lung cancer, testicular cancer, ovarian cancer and neuroblastoma. Marrow transplantation has been limited by the fact that many patients do not have HLA-identical siblings and very few have monozygotic twins. More recently, marrow transplants from HLA-nonidentical family members and even from unrelated donors have been successfully explored.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Marrow transplantation: the Seattle experience. 391 47

The complete amino acid sequence has been determined of a unique protein from a 55-years-old female with multiple myeloma associated with Fanconi syndrome. It existed in a monomer form with an apparent molecular weight of 10K daltons, and was consisted of 106 amino acid residues. The sequence was characteristic of the V-region of lambda light chains and was highly homologous with that of the first 106 residues of V lambda III subgroup. The presence of an intact light chain as well as a 13K daltons fragment, corresponding to the entire C-region, strongly suggests that the unique component is a catabolic product from the intact light chain rather than an aberrant product of synthesis.
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PMID:Complete amino acid sequence of a unique protein related to the variable domain of lambda light chain from a case with Fanconi syndrome. 391 18

Two adult cases of the Fanconi syndrome are described, in each of which there was abnormal urinary excretion of immunoglobulin kappa-chain. The significance of this finding is discussed in relation to the recognized association between multiple myeloma and the Fanconi syndrome.
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PMID:Adult Fanconi syndrome with monoclonal abnormality of immunoglobulin light chain. 601 86

The authors describe a 70-year-old woman with multiple myeloma and adult Fanconi syndrome. A monoclonal protein of IgA heavy-chain class and kappa light-chain class was demonstrable in the serum. Urine immunoelectrophoresis showed the presence of kappa light chains. Bone marrow aspirate showed increased plasma cells with large bundles of pink-staining Auer-rod-like crystals in their cytoplasm. These crystals failed to stain with Sudan black B, peroxidase, esterase, and PAS, but showed strong acid phosphatase and beta-glucuronidase positivity. Ultrastructural studies showed them to have a fibrillar and an unusual cross-striated pattern. Immunofluorescent studies showed strong IgA and kappa activity in the cytoplasm of the tumor cells, but the fluorescence was absent in the region of the crystals, which were identified easily by their negative birefringence. The authors interpret these observations to indicate that the intracytoplasmic crystals in this case are of lysosomal origin.
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PMID:Nature of intracytoplasmic crystalline inclusions in myeloma cells (morphologic, cytochemical, ultrastructural, and immunofluorescent studies). 619 1

First description of a case of early myeloma with monoclonal IgG, type kappa, anomaly plus Bence-Jones proteinuria and Fanconi's syndrome in a 67-year-old woman. Characteristic crystalline inclusion bodies were found in plasma cells of bone marrow and renal tissue.
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PMID:[Fanconi syndrome in an adult coincident with early myeloma with monoclonal IgG, type kappa, anomaly (author's transl)]. 625 69

A case of adult Fanconi syndrome is described in which there was urinary excretion of kappa light chains. After 13 years the patient developed overt myeloma. She also developed an adenocarcinoma of the colon and an adenocarcinoma of the parathyroid gland. These findings are discussed in relation to the known association between adult Fanconi syndrome, renal damage, and myeloma.
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PMID:Adult Fanconi syndrome progressing to multiple myeloma. 643 83

The case here reported of 72 year-old female with osteomalacia in whom an adult's Fanconi's syndrome with distal tubular involvement and a monoclonal IgG-lambda paraprotein were discovered. There was urinary excretion of lambda light chains without evidence of myeloma or amyloidosis. Such apparently unrelated entities might be subject to an unitary pathophysiological approach: the nephrotoxicity of light chains could cause a Fanconi's syndrome, which in turn would give rise to osteomalacia through phosphate depletion. It is noteworthy that in this patient the urinary excretion of light chains was of the lambda type, in contrast to similar cases described in the literature which presented mostly kappa chains. The likelihood of this patient developing myeloma or amyloidosis at a later stage is discussed.
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PMID:[Monoclonal IgG lambda paraprotein, Fanconi's syndrome of the adult, and osteomalacia (author's transl)]. 678 49

Six cases of multiple myeloma were studied regarding functions and pathology of the kidney. Glomerular changes were minimal, whereas tubular and interstitial changes were noted in all cases. Tubular cells were degenerative and atrophic in cases excreting more than 3 g BJP daily in the urine. Cases without BJP had only minor changes. The most severe impairment of renal function was found in a patient with Fanconi's syndrome, with hypercalcemia, calcium in the tubules, tubular atrophy and interstitial fibrosis. In attempting to correlate immunoglobulin abnormalities with renal histology, immunoperoxidase staining of immunoglobulin heavy and light chains were done on kidney sections. Glomerular and interstitial tissues gave a definitely negative reaction. In contrast, positive staining of light chain of corresponding type wrs found in the cytoplasma of the tubules and most tubular casts, with different degrees possibly in accord with the amounts of BJP.
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PMID:Localization of Bence Jones proteins in the kidney of myeloma patients. 680 48

We studied seven patients with idiopathic Bence Jones proteinuria (excretion greater than 1.0 g per 24 hours) who did not have acquired Fanconi's syndrome or an intact monoclonal immunoglobulin in the serum. None had evidence of overt multiple myeloma, of its variants, of systemic amyloidosis, or of malignant lymphoproliferative diseases when the proteinuria was recognized. In three of the seven patients, symptomatic multiple myeloma (associated with systemic amyloidosis in one) developed 8.8 to 21.1 years later. A fourth patient has asymptomatic myeloma and has not been treated. A fifth patient had an evolving myeloma at nine years but died of an unrelated cause. The remaining two patients still have apparently benign Bence Jones proteinuria after 7.7 and 12 years. Plasma-cell labeling indexes were low in all cases tested. This experience shows that although idiopathic Bence Jones proteinuria may remain stable for years, multiple myeloma or amyloidosis often develops, and consequently these patients must be kept under observation indefinitely.
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PMID:"Idiopathic" Bence Jones proteinuria: long-term follow-up in seven patients. 1208 18

Adult Fanconi syndrome has been described as a manifestation of a latent form of myeloma. Since benign monoclonal gammopathy (BMG) is a latent form of myeloma we studied ten patients with this disease with respect to the adult Fanconi syndrome. These patients were selected from a larger group with BMG because of urinary excretion of monoclonal light chains. In no instance could a complete Fanconi syndrome be shown, but one patient, who also had a diabetic nephropathy, had very high levels of urinary beta 2-microglobulins. Two additional patients showed minor features included in the adult Fanconi syndrome. BMG does not seem to be associated with an increased risk of developing adult Fanconi syndrome.
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PMID:Occurrence of adult Fanconi syndrome in benign monoclonal gammopathy. 746 11


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