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两名患有骨髓瘤病和高粘滞综合征患者体内的聚合单克隆IgA

Polymerized monoclonal IgA in tuo patients with myelomatosis and hyperviscosity syndrome.

作者信息

Virella G, Preto R V, Graça F

出版信息

Br J Haematol. 1975 Aug;30(4):479-87. doi: 10.1111/j.1365-2141.1975.tb01862.x.

Abstract

Two cases of multiple myeloma with IgA gammopathy and the syndrome of serum hyperviscosity are reported. A common feature of both patients was the production of large amounts of polymeric IgA (more than 50% of the monoclonal proteins existed as dimers or larger forms). However, similar large amounts of polymeric IgA were present in sera with only slightly raised viscosity, suggesting that factors such as the molecular configuration of the polymers or their sub-units may play a role in determining serum viscosity. The association of plasmapheresis and chemotherapy produced remarkable improvements of the symptoms attributable to increased serum viscosity, and 16 months after diagnosis one of the patients has survived relatively free of symptoms. The hyperviscosity syndrome is being diagnosed with increasing frequency in association with IgA multiple myeloma, and should be investigated in all cases with monoclonal proteins exceeding a concentration of 3-0 g/dl.

摘要

报告了两例伴有IgA丙种球蛋白病和血清高黏滞综合征的多发性骨髓瘤病例。两名患者的一个共同特征是产生大量聚合性IgA(超过50%的单克隆蛋白以二聚体或更大形式存在)。然而,在黏度仅略有升高的血清中也存在类似大量的聚合性IgA,这表明聚合物或其亚基的分子构型等因素可能在决定血清黏度中起作用。血浆置换和化疗联合应用使因血清黏度增加所致的症状有显著改善,诊断后16个月,其中一名患者存活且症状相对较轻。与IgA多发性骨髓瘤相关的高黏滞综合征的诊断频率越来越高,所有单克隆蛋白浓度超过3.0 g/dl的病例均应进行检查。

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