目的:评估白细胞来源的趋化性2(LECT2)的患病率,它的器官参与,以及它在种族偏见人群尸检中的临床关联。
方法:对诊断为淀粉样变性的个体的所有尸检组织进行重新评估,并对淀粉样蛋白轻链(AL)淀粉样变性进行分型,淀粉样蛋白A(AA)淀粉样变性,甲状腺素运载蛋白淀粉样变性(ATTR),免疫组织化学和白细胞趋化因子2淀粉样变性(ALECT2)。描述了器官受累并与其临床关联相关。
结果:在782例尸检中,27(3.5%)已确诊为淀粉样变性。其中,14(52%)对应于ALECT2,5(19%)对应于AL淀粉样变性,2(7%)到ATTR淀粉样变性,1(4%)到AA淀粉样变性,5(21%)为未确定型淀粉样变性。LECT2淀粉样蛋白沉积在肾脏中发现,肝脏,脾,脾大多数人的肾上腺。除了肾脏,在大多数受影响的器官中没有提示淀粉样蛋白沉积的临床迹象。LECT2淀粉样变性在任何情况下都与死亡原因无关。没有病例有心脏或大脑受累。在肾上腺和脾脏等器官中淀粉样蛋白沉积的潜在亚临床效应需要进一步研究。
结论:这项尸检研究证实了墨西哥人群中LECT2淀粉样变性的高患病率,经常在肾脏沉积淀粉样蛋白,肝脏,脾,脾和肾上腺.
To assess the prevalence of leukocyte cell-derived chemotactic 2 (LECT2), its organ involvement, and its clinical association in autopsies from an ethnically biased population.
The tissues from all autopsies of individuals diagnosed with amyloidosis were reassessed and typed for amyloid light chain (AL) amyloidosis, amyloid A (AA) amyloidosis, transthyretin amyloidosis (ATTR), and leukocyte chemotactic factor 2 amyloidosis (
ALECT2) by immunohistochemistry. Organ involvement was described and correlated with its clinical associations.
Of 782 autopsies, 27 (3.5%) had a confirmed diagnosis of amyloidosis. Of these, 14 (52%) corresponded to
ALECT2, 5 (19%) to AL amyloidosis, 2 (7%) to ATTR amyloidosis, 1 (4%) to AA amyloidosis, and 5 (21%) as undetermined-type amyloidosis. The LECT2 amyloid deposits were found in the kidneys, liver, spleen, and adrenal glands in most individuals. Except for the kidneys, there were no clinical signs suggestive of amyloid deposition in most of the affected organs. LECT2 amyloidosis was not associated with the cause of death in any case. No cases had heart or brain involvement. Potential subclinical effects of amyloid deposition in organs such as adrenal glands and spleen require further study.
This autopsy study confirms the high prevalence of LECT2 amyloidosis in the Mexican population, with frequent amyloid deposition in the kidneys, liver, spleen, and adrenal glands.