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骨髓瘤病与高黏滞综合征

Myelomatosis and the hyperviscosity syndrome.

作者信息

Preston F E, Cooke K B, Foster M E, Winfield D A, Lee D

出版信息

Br J Haematol. 1978 Apr;38(4):517-30. doi: 10.1111/j.1365-2141.1978.tb01077.x.

Abstract

Eleven patients are described in whom myelomatosis was complicated by the laboratory and clinical features of the hyperviscosity syndrome (HVS). The myeloma type was IgA in nine and IgG3 in two. In those patients with IgA myeloma the HVS was related to the presence of high molecular weight complexes in the serum. Remission of clinical features was obtained in all patients by plasma exchange. Clinical improvement coincided with reduction of whole blood viscosity and in those patients with IgA myeloma, with a parallel reduction of the high molecular weight complexes. The relationship between the IgA complexes and blood viscosity has been examined by physicochemical analysis of purified IgA monomer and polymers and evidence is presented to show that the IgA polymer has a higher intrinsic viscosity and axial ratio than the larger IgM molecule. The significance of these observations is discussed.

摘要

本文描述了11例骨髓瘤患者,这些患者的骨髓瘤合并了高黏滞综合征(HVS)的实验室及临床特征。其中9例为IgA骨髓瘤,2例为IgG3骨髓瘤。在IgA骨髓瘤患者中,HVS与血清中高分子量复合物的存在有关。所有患者通过血浆置换后临床症状均得到缓解。临床症状改善与全血黏度降低同时出现,在IgA骨髓瘤患者中,高分子量复合物也同时减少。通过对纯化的IgA单体和聚合物进行物理化学分析,研究了IgA复合物与血液黏度之间的关系,并提供证据表明IgA聚合物比更大的IgM分子具有更高的特性黏度和轴比。本文还讨论了这些观察结果的意义。

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