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一个冰岛家族中的巨球蛋白血症

Macroglobulinaemia in an Icelandic family.

作者信息

Björnsson O G, Arnason A, Gudmunosson S, Jensson O, Olafsson S, Valimarsson H

出版信息

Acta Med Scand. 1978;203(4):283-8. doi: 10.1111/j.0954-6820.1978.tb14874.x.

Abstract

Macroglobulinaemia in an Icelandic family is presented. A woman had Waldenström's macroglobulinaemia, and two of her brothers had monoclonal macroglobulinaemia of the benign form. One was asymptomatic, but the other had polyneuropathy and IgM deposits in peripheral nerves. A third brother of these siblings died of a lymphoreticular disease, which presented with a widespread neuropathy. A second sister had polyclonal increase in serum IgA and two other brothers of this sibship had IgM slightly elevated. A study of all descendants (45 in all and 19 spouses) revealed seven individuals with elevated IgM levels. No other immunoglobulin abnormalities were detected.

摘要

本文介绍了一个冰岛家族中的巨球蛋白血症病例。一名女性患有华氏巨球蛋白血症,她的两个兄弟患有良性单克隆巨球蛋白血症。其中一人无症状,另一人患有多发性神经病且外周神经有IgM沉积。这些兄弟姐妹中的第三个兄弟死于一种淋巴网状疾病,伴有广泛的神经病变。第二个姐妹血清IgA呈多克隆性增加,该家族的另外两个兄弟IgM略有升高。对所有后代(共45人及19名配偶)的研究发现7人IgM水平升高。未检测到其他免疫球蛋白异常。

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