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多发性骨髓瘤、冷球蛋白血症和黄瘤病。两名患者的不同临床和生化综合征。

Multiple myeloma, cryoglobulinemia and xanthomatosis. Distinct clinical and biochemical syndromes in two patients.

作者信息

Wilson D E, Floweres C M, Hershgold E J, Eaton R P

出版信息

Am J Med. 1975 Nov;59(5):721-9. doi: 10.1016/0002-9343(75)90233-8.

Abstract

Studies were carried out in two patients with multiple myeloma (immunoglobulin G, [IgG], K light chain), cryoglobulinemia and xanthomatosis with clinical features and lipid transport abnormalities which were quite different. One patient had nodular xanthomatosis and lipemia with delayed triglyceride and apolipoprotein removal. In vivo heparin resistance was present and heparin-paraprotein interaction was shown in vitro. The lipoprotein removal defect may have been due to impaired uptake of the "remnants" of glyceride-rich lipoproteins. Abnormalities were found both in primary platelet aggregation and in the platelet release reaction. The second patient had diffuse plane xanthomatosis with normal lipids. An orange cryoprecipitate contained IgG, beta- and prebeta lipoproteins, albumin, carotenoids and about half of the serumcholesterol. Triglyceride turnover was normal. These observations show that M-proteins may interfere with lipid transport by at least two mechanisms and illustrate the clinical diversity of xanthomatous myeloma.

摘要

对两名患有多发性骨髓瘤(免疫球蛋白G,[IgG],κ轻链)、冷球蛋白血症和黄瘤病的患者进行了研究,这两名患者的临床特征和脂质转运异常情况差异很大。一名患者患有结节性黄瘤病和脂血症,甘油三酯和载脂蛋白清除延迟。体内存在肝素抵抗,体外显示肝素与副蛋白相互作用。脂蛋白清除缺陷可能是由于富含甘油酯的脂蛋白“残粒”摄取受损所致。在原发性血小板聚集和血小板释放反应中均发现异常。第二名患者患有弥漫性扁平黄瘤病,血脂正常。一种橙色冷沉淀物含有IgG、β和前β脂蛋白、白蛋白、类胡萝卜素和约一半的血清胆固醇。甘油三酯周转率正常。这些观察结果表明,M蛋白可能至少通过两种机制干扰脂质转运,并说明了黄瘤性骨髓瘤的临床多样性。

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