系统性淀粉样变性患者软脑膜、皮质、脉络丛和垂体血管中的淀粉样蛋白。

Amyloid protein of vessels in leptomeninges, cortices, choroid plexuses, and pituitary glands from patients with systemic amyloidosis.

作者信息

Ishihara T, Nagasawa T, Yokota T, Gondo T, Takahashi M, Uchino F

机构信息

First Department of Pathology, Yamaguchi University School of Medicine, Ube, Japan.

出版信息

Hum Pathol. 1989 Sep;20(9):891-5. doi: 10.1016/0046-8177(89)90102-0.

DOI:10.1016/0046-8177(89)90102-0

PMID:2673980

Abstract

The cerebrum, cerebellum, and choroid plexuses from 16 patients with systemic amyloidosis, and the pituitary glands from 14 of these patients, were investigated histologically and immunohistochemically. Cerebrovascular amyloid (CVA) was found in the leptomeninges and cortices of six patients with systemic amyloidosis, including two patients with amyloid A protein (AA) amyloidosis related to serum amyloid A protein, one with AL amyloidosis related to immunoglobulin light chain (AL), two with familial type I amyloidotic polyneuropathy (FAP), and one with senile systemic amyloidosis (SSA). CVA protein from two patients with FAP reacted with anti-human prealbumin antibody similar to that of the visceral organs of these two patients. CVA in SSA reacted with anti-human prealbumin antibody and anti-beta protein antibody. Vascular amyloid was frequently noted in the pituitary glands and choroid plexuses of patients with systemic amyloidosis, and was found to be identical to that in the visceral organs (heart, kidney, and intestine) of these patients. CVA in the leptomeninges and cortices from two patients with AA amyloidosis and one with AL amyloidosis reacted with anti-beta protein monoclonal antibody but not with anti-human AA monoclonal antibody, anti-human A lambda antisera, and anti-human A kappa antisera. We suggest that amyloid proteins of AA and AL amyloidosis do not readily accumulate in the vessels in the leptomeninges and cortices even though the proteins circulate, and that beta protein is not derived from a serum precursor.

摘要

对16例系统性淀粉样变性患者的大脑、小脑和脉络丛，以及其中14例患者的垂体进行了组织学和免疫组织化学研究。在6例系统性淀粉样变性患者的软脑膜和皮质中发现了脑血管淀粉样变性（CVA），其中包括2例与血清淀粉样蛋白A相关的淀粉样蛋白A（AA）淀粉样变性患者、1例与免疫球蛋白轻链（AL）相关的AL淀粉样变性患者、2例家族性I型淀粉样多神经病（FAP）患者和1例老年系统性淀粉样变性（SSA）患者。2例FAP患者的CVA蛋白与抗人前白蛋白抗体反应，类似于这2例患者内脏器官的反应。SSA中的CVA与抗人前白蛋白抗体和抗β蛋白抗体反应。系统性淀粉样变性患者的垂体和脉络丛中经常发现血管淀粉样变性，并且发现其与这些患者的内脏器官（心脏、肾脏和肠道）中的血管淀粉样变性相同。2例AA淀粉样变性患者和1例AL淀粉样变性患者的软脑膜和皮质中的CVA与抗β蛋白单克隆抗体反应，但不与抗人AA单克隆抗体、抗人Aλ抗血清和抗人Aκ抗血清反应。我们认为，AA和AL淀粉样变性的淀粉样蛋白即使循环，也不容易在软脑膜和皮质的血管中积聚，并且β蛋白不是来源于血清前体。