Saba Yasir MBBS, Zongming Eric Chen MD, PhD, Chris Hartley MD, Lizhi Zhang MD, Michael Torbenson MD
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引用次数: 0
Abstract
The classic findings have been well described for light-chain amyloid involving the liver. In addition to light chain, however, many additional proteins are now known to be amyloidogenic and can involve the liver. A total of 58 surgical pathology specimens with amyloid deposits were analyzed for patterns of amyloid deposition, including amyloid from light chain lambda (N = 17), light chain kappa (N = 15), transthyretin (N = 15), serum amyloid A (N = 4), apolipoprotein A1 (N = 4), fibrinogen alpha (N = 2), LECT2 (N = 1). Amyloid deposits predominately targeted the liver vasculature, including the walls of the hepatic arteries, portal veins, and sinusoids. While there was overlap, light chain amyloid predominately involved the sinusoids, while transthyretin amyloid predominately targeted the hepatic arteries, especially the larger ones in the hilum and larger portal tracts. Serum amyloid A formed nodular deposits that started in the portal vasculature but then extended into the portal tract stroma, leading to large, bulbous, portal-based amyloid deposits. Apolipoprotein A amyloid also formed large portal-based nodules. Fibrinogen was mild and subtle on H&E and predominately affected portal veins. Amyloid deposits in hilar nerves were prominent with amyloid light chain, transthyretin, and apolipoprotein A1. In conclusion, the histology of hepatic amyloid is diverse and shows several distinct clusters of findings that can aide in recognition in surgical pathology specimens.
关于轻链淀粉样蛋白累及肝脏的经典研究结果已经有了很好的描述。然而,除轻链外,目前已知还有许多蛋白质可产生淀粉样蛋白,并可累及肝脏。我们对58例淀粉样沉积的外科病理标本进行了淀粉样沉积模式分析,包括轻链λ(17例)、轻链卡帕(15例)、转甲状腺素(15例)、血清淀粉样蛋白A(4例)、脂蛋白A1(4例)、纤维蛋白原α(2例)和LECT2(1例)。淀粉样蛋白沉积主要针对肝脏血管,包括肝动脉、门静脉和肝窦壁。虽然存在重叠,但轻链淀粉样蛋白主要累及肝窦,而转甲状腺素淀粉样蛋白主要累及肝动脉,尤其是肝门和较大门静脉的较大动脉。血清淀粉样蛋白 A 形成的结节状沉积物始于门静脉血管,但随后扩展到门静脉道基质,导致门静脉淀粉样沉积物呈大球状。载脂蛋白 A 淀粉样蛋白也形成了大的门静脉结节。纤维蛋白原在H&E上表现轻微,主要累及门静脉。肝神经中的淀粉样蛋白沉积突出,包括淀粉样轻链、转甲状腺素和载脂蛋白A1。总之,肝淀粉样变的组织学表现多种多样,并显示出几组不同的发现,有助于在手术病理标本中进行识别。
期刊介绍:
Human Pathology is designed to bring information of clinicopathologic significance to human disease to the laboratory and clinical physician. It presents information drawn from morphologic and clinical laboratory studies with direct relevance to the understanding of human diseases. Papers published concern morphologic and clinicopathologic observations, reviews of diseases, analyses of problems in pathology, significant collections of case material and advances in concepts or techniques of value in the analysis and diagnosis of disease. Theoretical and experimental pathology and molecular biology pertinent to human disease are included. This critical journal is well illustrated with exceptional reproductions of photomicrographs and microscopic anatomy.