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轨道沃尔登斯特罗姆巨型球蛋白血症与血管病变性偏蛋白沉积:一个病例报告

Terence Ang^1,2, Abdullah Almater^2,3,4, Craig James⁵

¹Discipline of Ophthalmology and Visual Sciences, The University of Adelaide.

Ophthalmic plastic and reconstructive surgery

|December 19, 2025

概括

沃尔登斯特罗姆巨型球蛋白血症 (WM) 很少透到轨道上. 这个案例突出显示了eosinophilic hyaline paraprotein在轨道上的沉积,导致视力丧失而没有超粘性综合征,为罕见的WM眼部并发症提供了洞察力.

科学领域:

眼科医生眼科眼科
血液学血液学血液学
在瘤学瘤学.

背景情况:

沃尔登斯特罗姆巨型球蛋白血症 (WM) 的眼部表现通常涉及由于高粘度而导致的视网膜病变.
通过淋巴细胞沉积的轨道和眼部附录透在WM中很罕见.

研究的目的:

报告一个独特的WM轨道透病例,呈现暂时视力丧失.
在没有超粘度综合征的情况下,描述导致视力丧失的沉积物质的性质.

主要方法:

一个66岁的女性WM和轨道透病例报告.
轨道活检的分析,包括组织病理学,特殊染色 (PAS,刚果红,水晶紫) 和IgM免疫组织化学.

主要成果:

患者经历了循环的短暂视力丧失,原因是eosinophilic hyaline paraprotein在轨道上的血管病态沉积.
活检揭示了酸细胞和血管壁的eosinophilic氨酸物质,对PAS和IgM阳性,但对粉样蛋白阴性.
与卡帕轻链限制相关的低级淋巴细胞B细胞淋巴瘤证实了WM透.

结论:

可能会发生WM的轨道透,呈现出独特的异位病理学发现,即eosinophilic hyaline paraprotein沉积.
这种沉积可以导致视力损失,独立于超粘度综合征.
这些发现扩大了沃尔登斯特罗姆巨型球蛋白血症眼睛表现的范围.

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