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IgA 骨髓瘤合并骨骨折及高黏滞综合征——一例尸检病例报告

IgA myeloma complicated by fractures of the bones and hyperviscosity syndrome--report of an autopsied case.

作者信息

Tojo K, Morita A, Miki T, Morii H, Mitsuhashi T, Hashimoto R, Kadoya Y

机构信息

2nd Department of Internal Medicine, Osaka City University Medical School.

出版信息

Osaka City Med J. 1990 May;36(1):61-70.

PMID:2385441
Abstract

A case of IgA (kappa type) myeloma complicated by fractures of the bones and hyperviscosity syndrome is presented. A 56-year-old woman who had been followed as an outpatient with diabetes mellitus for about 16 years, developed multiple myeloma. She also showed symptoms and signs of hyperviscosity syndrome; hemorrhagic diathesis, blurred vision and episodes of transient ischemic attacks of the brain, and fractures of the bones by small powers of trauma. At autopsy, almost all bones showed infiltration of multiple myeloma of IgA-kappa type and severe osteoporosis accompanied with proliferation of osteoclasts. The association of IgA myeloma with hyperviscosity syndrome and/or bone destruction was discussed. The "serum hyperviscosity syndrome" has been described clinically as the triad of bleeding, visual signs and symptoms, and neurologic manifestations. And this syndrome has been associated frequently with macroglobulinemia of Waldenström, occasionally with immunoglobulin (Ig) G myeloma, rarely with IgA myeloma, and with other dysproteinemia. Myeloma is also characterized by extensive bone destruction and is accompanied by susceptibility to fracture, although Waldenström's macroglobulinemia, acute leukemia or chronic leukemia are rarely associated with bone resorption. The present report describes a patient with IgA myeloma complicated by hyperviscosity syndrome and multiple bone fractures.

摘要

本文报告一例IgA(κ型)骨髓瘤合并骨骨折及高黏滞综合征的病例。一名56岁女性,作为糖尿病门诊患者随访约16年,后发展为多发性骨髓瘤。她还表现出高黏滞综合征的症状和体征,包括出血倾向、视力模糊和短暂性脑缺血发作,以及轻微外力导致的骨骨折。尸检时,几乎所有骨骼均显示IgA-κ型多发性骨髓瘤浸润及严重骨质疏松,并伴有破骨细胞增殖。文中讨论了IgA骨髓瘤与高黏滞综合征和/或骨破坏的关联。“血清高黏滞综合征”临床上表现为出血、视觉症状和体征以及神经学表现三联征。该综合征常与华氏巨球蛋白血症相关,偶尔与免疫球蛋白(Ig)G骨髓瘤有关,很少与IgA骨髓瘤相关,也与其他异常蛋白血症有关。骨髓瘤还以广泛的骨破坏为特征,并伴有骨折易感性,尽管华氏巨球蛋白血症、急性白血病或慢性白血病很少与骨吸收相关。本报告描述了一名患有IgA骨髓瘤合并高黏滞综合征和多发性骨折的患者。

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