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IgA 骨髓瘤中副蛋白聚合与临床特征的关系

Relationship between paraprotein polymerization and clinical features in IgA myeloma.

作者信息

Roberts-Thomson P J, Mason D Y, MacLennan I C

出版信息

Br J Haematol. 1976 May;33(1):117-30. doi: 10.1111/j.1365-2141.1976.tb00978.x.

Abstract

The clinical manifestation fo IgA multiple myeloma are usually not considered distinguishable from those of IgG myeloma despite the fact that IgA differs from IgG in several characteristics, particularly molecular size heterogeneity. The clinical and laboratory features of 25 patients with IgA myeloma seen during a 5 year period are presented. The degree of paraprotein polymerization was observed to vary greatly in these patients but remained chronologically constant in six individuals studied on several occasions over this period. The patients were divided into two groups on the basis of the degree of paraprotein polymerization. The first group comprised those patients in whom the IgA paraprotein was greater than 50% polymerized, whilst in the second group the paraprotein was predominantly monomeric. No clinical or pathological differences were seen between the 'polymeric' and 'monomeric' groups of myeloma apart from that directly attributable to the physicochemical effect of the paraprotein polymerization. Thus, five patients out of 11 of the 'polymeric' group had developed the hyperviscosity syndrome, whilst no patients in the 'monomeric' group had developed this complication. The concentration of the paraprotein during the course of the disease was comparable in both groups. This syndrome is considered to be relatively common in IgA myeloma and adds to the morbidity and mortality of the disease. Its anticipation and treatment may improve the quality and survival of patients likely to develop this complication.

摘要

尽管IgA在几个特征上与IgG不同,特别是分子大小的异质性,但IgA多发性骨髓瘤的临床表现通常被认为与IgG骨髓瘤没有区别。本文介绍了在5年期间观察到的25例IgA骨髓瘤患者的临床和实验室特征。在这些患者中观察到副蛋白聚合程度差异很大,但在这期间多次研究的6名个体中,其随时间保持恒定。根据副蛋白聚合程度将患者分为两组。第一组包括那些IgA副蛋白聚合度大于50%的患者,而第二组副蛋白主要为单体。除了直接归因于副蛋白聚合的物理化学效应外,骨髓瘤的“聚合”组和“单体”组之间未观察到临床或病理差异。因此,“聚合”组的11名患者中有5名出现了高粘滞综合征,而“单体”组中没有患者出现这种并发症。两组患者疾病过程中副蛋白的浓度相当。这种综合征在IgA骨髓瘤中被认为相对常见,并增加了该疾病的发病率和死亡率。对其进行预测和治疗可能会改善可能发生这种并发症的患者的生活质量和生存率。

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