Normolipidemic planar xanthomatosis associated with benign monoclonal gammopathy.
Normolipidemic planar xanthomatosis associated with benign monoclonal gammopathy.
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DOI：
10.1016/0026-0495(81)90073-1
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发表时间：
1981-09
期刊：
影响因子：
--
通讯作者：
E. Groszek;J. Abrams;S. Grundy
中科院分区：
文献类型：
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作者：
E. Groszek;J. Abrams;S. Grundy
本报告描述了一个病人与先进的平面黄瘤病和良性单克隆丙种球蛋白病谁有正常的血浆浓度的胆固醇和甘油三酯（TG）。极低密度脂蛋白（VLDL）、中密度脂蛋白（IDL）和乳糜微粒电泳迁移率降低，低密度脂蛋白（LDL）和高密度脂蛋白（HDL）电泳迁移率无变化。该患者的疾病与2年内血浆TG和VLDL-TG的进行性下降相关。他似乎还出现VLDL-TG和乳糜微粒清除率增加。含TG脂蛋白代谢的这些变化不伴随胆固醇或胆汁酸合成的改变。该患者的观察结果使我们得出结论，他的正常至低水平的VLDL-TG可以通过增强的组织摄取和增加的VLDL-TG脂解来解释。这些发现最可能的解释是，患者的富含TG的脂蛋白和异常的单克隆γ球蛋白之间存在免疫复合物。该患者是另一个扩大的受试者群体的例子，其中黄瘤病可以在没有高脂血症的情况下发展。
This report describes a patient with advanced planar xanthomatosis and benign monoclonal gammopathy who had normal plasma concentrations of cholesterol and triglycerides (TG). Decreased electrophoretic mobilities of very low density lipoprotein (VLDL), intermediate density lipoproteins (IDL) and chylomicrons were found. No changes in electrophoretic mobility were found for low density lipoprotein (LDL) and high density lipoproteins (HDL). The patient's disease was associated with a progressive decline in plasma-TG and VLDL-TG over a 2 yr period. He also appeared to develop an increased rate of clearance of VLDL-TG and chylomicrons. These changes in the metabolism of TG-containing lipoproteins were unaccompanied by alterations in the synthesis of either cholesterol or bile acids. The observations in this patient led us to the conclusion that his normal to low levels of VLDL-TG could be explained by both enhanced tissue uptake and increased lipolysis of VLDL-TG. The most likely explanation for these findings is that the patient had immune complexes between his TG-rich lipoproteins and an abnormal monoclonal gamma globulin. This patient is another example of an expanding group of subjects in whom xanthomatosis can develop without hyperlipidemia.