Globular Hepatic Amyloid: An Early Stage in the Pathway of Amyloid Formation: A Study of 20 New Cases
Globular Hepatic Amyloid: An Early Stage in the Pathway of Amyloid Formation: A Study of 20 New Cases
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球状肝淀粉样蛋白：淀粉样蛋白形成途径的早期阶段：20 个新病例的研究
DOI：
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发表时间：
2007
影响因子：
5.6
通讯作者：
Z. Goodman
中科院分区：
文献类型：
--
作者：
H. Makhlouf;Z. Goodman
只有25例球形肝淀粉样变性的报告，大多数是从20世纪80年代初。我们回顾了自1970年以来在武装部队病理研究所发现的208例肝淀粉样变性中的20例肝球状淀粉样变性的临床、组织病理学和免疫组化特征。14例（70%）为男性，6例为女性，中位年龄为67岁（范围：40 - 92岁）。超过一半的患者是西班牙裔。20例患者中有10例被诊断为系统性淀粉样变性。组织学上，所有病例均显示圆形至椭圆形，有时为层压小球，直径1至40 μm，6例有证据表明从球形过渡到更常见的线性形式。在所有20例中，刚果红和/或天狼星红将小球染成红色，并在偏振光下显示苹果绿色双折射。15/20例合并肝门脉及肝实质受累，5例仅合并肝窦沉积。血管沉积非常常见，超过3/4的病例主要表现为小静脉周围的淀粉样蛋白，同时累及终末肝小静脉和门静脉分支。少数肝细胞内小球存在于一半的情况下。总之，肝淀粉样变性很少以球状形式发生，细胞内淀粉样球和球状至线状沉积模式的过渡形式的发现表明这是系统性淀粉样变性累及肝脏的早期形式。
Only 25 cases of globular hepatic amyloidosis have been reported, mostly from the early 1980s. We reviewed clinical, histopathologic, and immunohistochemical features of 20 cases of hepatic globular amyloid out of 208 cases of liver amyloidosis seen at the Armed Forces Institute of Pathology since 1970. Fourteen (70%) were men and 6 were women with a median age of 67 years (range, 40 to 92 y). More than half of the patients were Hispanic. Ten of 20 patients were diagnosed with systemic amyloidosis. Histologically, all cases revealed round to oval-shaped sometimes laminated globules, 1 to 40 μm in diameter, and 6 cases had evidence of transition from globular to the more usual linear form. In all 20 cases, Congo red and/or Sirius red stained the globules red and showed an apple green birefringence under polarized light. Portal tracts and parenchyma were involved in 15/20 the cases, and sinusoidal deposition alone in 5 cases. Vascular deposition was very common with more than 3/4 of the cases showing mainly perivenular amyloid with both the terminal hepatic venules and portal vein branches being equally involved. A few intrahepatocellular globules were present in half of the cases. In conclusion, hepatic amyloidosis can rarely occur as a globular form, and the finding of intracellular amyloid globules and transitional forms of globular to linear patterns of deposition suggest that this is an early form of hepatic involvement by systemic amyloidosis.
DOI：
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发表时间：
1984
期刊：
The American journal of pathology
影响因子：
--
作者：
Chopra,S;Rubinow,A;Koff,RS;Cohen,AS
通讯作者：
Cohen,AS