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多发性骨髓瘤中的系统性κ轻链沉积与淀粉样变性:新的形态学观察

Systemic kappa light chain deposition and amyloidosis in multiple myeloma: novel morphological observations.

作者信息

Kirkpatrick C J, Curry A, Galle J, Melzner I

出版信息

Histopathology. 1986 Oct;10(10):1065-76. doi: 10.1111/j.1365-2559.1986.tb02543.x.

Abstract

Light and transmission electron microscopic studies as well as immunohistochemical investigations were performed on a case of multiple myeloma in a 40-year-old female with systemic kappa light chain deposition. The latter took the form of extensive deposits in the liver, spleen and bone marrow. Amyloid was not found in these deposits, although it was present in the wall of branches of the portal vein. Conversely, amyloid but not light chain deposition was found in the tongue, synovial membrane from the knee and myocardium. The kidney was the only organ to show both amyloid and light chain deposits intimately associated with each other in and around tubules and collecting ducts. Ultrastructurally the light chain deposits were seen to be biphasic, having two granular components of different electron density. In the spleen and bone marrow, but not in the liver, the kappa light chain deposits were surrounded by multinucleated giant cells. This is the first report of light chain deposition disease associated with a foreign body type of giant cell reaction. The morphological variability of organ involvement suggests that the molecular structure and conformation of the deposited light chains are heterogeneous and may reflect a broad spectrum of metabolism of these deposits in various organs.

摘要

对一名40岁患有系统性κ轻链沉积的女性多发性骨髓瘤病例进行了光镜和透射电镜研究以及免疫组化研究。后者表现为在肝脏、脾脏和骨髓中广泛沉积。在这些沉积物中未发现淀粉样蛋白,尽管在门静脉分支壁中存在淀粉样蛋白。相反,在舌头、膝关节滑膜和心肌中发现了淀粉样蛋白,但未发现轻链沉积。肾脏是唯一在肾小管和集合管内及周围同时显示淀粉样蛋白和轻链沉积物紧密相关的器官。超微结构显示轻链沉积物呈双相,有两个不同电子密度的颗粒成分。在脾脏和骨髓中,而非肝脏中,κ轻链沉积物被多核巨细胞包围。这是与异物型巨细胞反应相关的轻链沉积病的首次报告。器官受累的形态学变异性表明,沉积轻链的分子结构和构象是异质性的,可能反映了这些沉积物在各个器官中的广泛代谢谱。

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