Feussner G, von Hodenberg E, Ziegler R
Medizinische Klinik, Universität Heidelberg.
Klin Wochenschr. 1990 May 17;68(10):526-32. doi: 10.1007/BF01648250.
We studied a 58-year-old woman with severe therapy-refractory hyperlipidemia, xanthomatosis, and multiple myeloma (immunoglobulin A, lambda light chain). The lipid disorder became evident about half a year prior to the expression of myelomatosis. Clinical symptoms were similar to those found in classical type III hyperlipoproteinemia but the underlying metabolic defect was different from the one described in this primary dyslipoproteinemia. The patient has the heterozygous apolipoprotein E3/2 phenotype and her VLDL-cholesterol/serum-triglyceride ratio is unusually low at 0.05. Evidence is given that the hyperlipoproteinemia is due to an impaired catabolism of intermediate density lipoproteins probably because of a reduced hepatic triglyceride lipase activity.
我们研究了一名58岁女性，她患有严重的难治性高脂血症、黄瘤病和多发性骨髓瘤（免疫球蛋白A，λ轻链）。脂质紊乱在骨髓瘤病表现出来之前约半年就已明显。临床症状与经典III型高脂蛋白血症相似，但潜在的代谢缺陷与这种原发性血脂蛋白异常中所描述的不同。该患者具有杂合子载脂蛋白E3/2表型，其极低密度脂蛋白胆固醇/血清甘油三酯比值异常低，为0.05。有证据表明，高脂蛋白血症是由于中密度脂蛋白分解代谢受损，可能是因为肝脏甘油三酯脂肪酶活性降低。
