[Pulmonary amyloidosis and non-amyloid immunoglobulin deposits].
[Pulmonary amyloidosis and non-amyloid immunoglobulin deposits].
复制标题
[肺淀粉样变性和非淀粉样免疫球蛋白沉积]。
DOI：
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发表时间：
2008
影响因子：
0.6
通讯作者：
J. Cordier
中科院分区：
文献类型：
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作者：
J. Cordier
淀粉样变性的组织病理学特征为纤维状淀粉样蛋白的组织沉积，特别是被刚果红染色，在偏振光下呈双折射。这一特征与β-折叠结构构型有关，β-折叠结构构型是淀粉样变性的共同特征，淀粉样变性可能具有20多种不同的蛋白质前体。最常见的是AL淀粉样变性，它是免疫球蛋白来源的。它可能是器官限制性的，或全身性的（主要累及心脏）。局限性支气管肺淀粉样变性，通常为AL，可表现为气管支气管沉积物或实质结节或肿块。弥漫性间质性肺淀粉样变性的临床表现是罕见的，通常与系统性AL淀粉样变性和沉积物涉及肺泡毛细血管气体交换区。淀粉样变性也可表现为肺动脉高压、肺门和纵隔淋巴结肿大或胸膜受累。AL淀粉样变性可能与肺淋巴瘤局部相关。偶尔也可出现非纤维状、刚果红阴性免疫球蛋白沉积，表现为肺实质结节或囊肿。特殊的免疫球蛋白沉积物可能显示细胞或细胞外晶体结构。
The amyloidoses are characterised histopathologically by the tissue deposition of fibrillar amyloid, specifically stained by Congo red and birefringent under polarised light. This characteristic is linked to a beta-folded structural configuration that is the common denominator of the amyloidoses which may have more than twenty distinct protein precursors. The most common is AL amyloidosis which is of immunoglobulin origin. It may be organ limited, or systemic (with predominant cardiac involvement). Limited bronchopulmonary amyloidosis, usually AL, may manifest itself as either tracheobronchial deposits or parenchymal nodules or masses. Diffuse interstitial pulmonary amyloidosis with clinical manifestations is rare and usually associated with systemic AL amyloidosis and deposits involving the alveolar-capillary gas exchange zone. Amyloidosis may also manifest itself as pulmonary hypertension, amyloid hilar and mediastinal adenopathy or pleural involvement. AL amyloidosis may be associated locally with pulmonary lymphoma. Occasionally, non-fibrillar, Congo red-negative, immunoglobulin deposits may occur, presenting as parenchymal pulmonary nodules or cysts. Exceptionally immunoglobulin deposits may show a cellular or extra-cellular crystalline structure.