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 investigated the metabolism of intermediate-density lipoproteins (IDL [1.006 to 1.019 g per milliliter]) and low-density lipoproteins (LDL [1.019 to 1.063 g per milliliter]) in two men with Type III hyperlipoproteinemia associated with myelomatosis. In vivo kinetic studies using radiolabeled autologous lipoproteins demonstrated a greatly reduced fractional catabolic rate of IDL, relative to control values (patients vs. normal, 0.006 and 0.025 per hour vs. 0.20 +/- 0.08 per hour [mean +/- S.E.M]) and a greatly prolonged IDL-to-LDL conversion time (45 and 17 hours vs. 5.4 +/- 1.6 hours). In studies in vitro, LDL from both patients failed to bind to the LDL receptor of normal blood lymphocytes, whereas LDL from subjects with familial Type III hyperlipoproteinemia bound normally to the receptor. In one patient immunoglobulin was shown to be associated with IDL and LDL. Thus, hyperlipoproteinemia reflected an impaired metabolism of IDL, probably secondary to the binding of immunoglobulin to the lipoproteins. A similar impairment of receptor-mediated LDL catabolism did not elevate the plasma LDL concentration because of the low IDL-to-LDL conversion rate.
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PMID:Myelomatosis with type III hyperlipoproteinemia: clinical and metabolic studies. 628 45

Type III hyperlipidemia is a rare metabolic disorder characterized by elevated plasma concentrations of cholesterol and triglycerides. In subjects homozygous for the isoform E2 of apoprotein E, the disease becomes manifest when other factors that interfere with normal lipoprotein metabolism are present. Multiple myeloma has also been found to be associated with type III hyperlipidemia. We report a case with the typical manifestations of the disease (hyperlipidemia and palmar xanthoma) in whom the family history and blood analyses excluded pathologies potentially interfering with lipid metabolism. On electrophoresis of serum proteins, a monoclonal peak was detected. The patient was homozygous for the isoform E2 of the apoprotein E. Further blood analyses, bone marrow and roentgen examinations enabled the diagnosis of monoclonal gammopathy of undetermined origin. The association of type III hyperlipidemia with monoclonal gammopathy might be casual, although only the characterization of the antigenic determinants toward which the monoclonal antibodies are directed could be conclusive. The presence of several family members homozygous for the isoform E2, but without the clinical and biochemical characteristics of type III hyperlipidemia, and the poor response to diet and drug therapy suggest that gammopathy may play role in determining hyperlipidemia.
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PMID:[Dyslipoproteinemia and monoclonal gammopathy: a case report]. 899 66