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高黏滞综合征的研究。II. 巨球蛋白血症。

Studies of the hyperviscosity syndrome. II. Macroglobulinemia.

作者信息

MacKenzie M R, Babcock J

出版信息

J Lab Clin Med. 1975 Feb;85(2):227-34.

PMID:803540
Abstract

Thirty-four patients with macroglobulinemia were studied for the incidence of hyperviscosity syndrome (HVS) and the circumstances in which this complication occurred. The following were evaluated: total serum protein, quantity of IgM paraprotein, and relative serum viscosity. These measurements were coupled with physical-chemical studies of isolated IgM proteins, including molecular weight and evaluation of molecular shape, by determination of intrinsic viscosity, viscosity increment, ratio of frictional coefficient, and coefficient of concentration dependence. It was found that relative serum viscosity values in the symptomatic range (above 6.0) were present in 38 per cent of the patients and were usually associated with IgM values greater than 5.0 gm. per 100 ml. All IgM proteins studied were large, hydrodynamically active molecules. A patient who developed HVS with IgM values below 3.5 gm. per 100 ml. had markedly asymmetrical molecules. The principal factors in the pathogenesis of this syndrome in macroglobulinemia are the concentration and molecular shape of IgM. Additional factors include protein-protein interactions between IgM and serum constituents.

摘要

对34例巨球蛋白血症患者进行了高黏滞综合征(HVS)发病率及该并发症发生情况的研究。评估了以下指标:血清总蛋白、IgM副蛋白量及相对血清黏度。这些测量结果与对分离出的IgM蛋白的物理化学研究相结合,包括通过测定特性黏度、黏度增量、摩擦系数比及浓度依赖性系数来评估分子量和分子形状。结果发现,38%的患者存在症状性范围内(高于6.0)的相对血清黏度值,且通常与IgM值大于5.0克/100毫升相关。所有研究的IgM蛋白都是大的、具有流体动力学活性的分子。一名IgM值低于3.5克/100毫升却发生HVS的患者,其分子具有明显的不对称性。巨球蛋白血症中该综合征发病机制的主要因素是IgM的浓度和分子形状。其他因素包括IgM与血清成分之间的蛋白质-蛋白质相互作用。

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