Tariq Hamza, Sharkey Francis E
1 University of Texas Health Science Center at San Antonio, TX, USA.
Int J Surg Pathol. 2018 May;26(3):271-275. doi: 10.1177/1066896917735893. Epub 2017 Oct 11.
Amyloidosis caused by deposition of leukocyte cell-derived chemotaxin-2 amyloidosis (ALECT2) is the most recently described form of systemic amyloidosis and has quickly emerged as a common and possibly underdiagnosed cause of slowly declining renal function, particularly in patients of Hispanic ancestry. We describe the autopsy findings in a 70-year-old Hispanic woman who died of metastatic lung adenocarcinoma and was incidentally found to have extensive amyloid deposition in the kidneys, liver, spleen, adrenal glands, small intestine, gallbladder, lungs, bilateral ovaries, and uterus. The type of amyloid was confirmed to be ALECT2 by mass spectrometry. The pattern of amyloid deposition in the kidneys and the liver was typical for what has been described for ALECT2. In addition, a unique pattern of amyloid deposition was observed in spleen, adrenal glands, small intestine, gallbladder, lungs, ovaries, and uterus. The pattern of amyloid deposition in ALECT2 is distinct from amyloid A and amyloid light-chain and needs to be recognized to avoid misdiagnosis as amyloid light-chain or amyloid A amyloidosis. After recognition, an accurate diagnosis by mass spectrometry and/or immunohistochemical staining is essential to guide treatment and avoid toxic therapies.
由白细胞衍生趋化因子-2淀粉样变性(ALECT2)沉积引起的淀粉样变性是最近描述的一种全身性淀粉样变性形式,已迅速成为肾功能缓慢下降的常见且可能诊断不足的原因,尤其是在西班牙裔血统的患者中。我们描述了一名70岁西班牙裔女性的尸检结果,该女性死于转移性肺腺癌,尸检时偶然发现其肾脏、肝脏、脾脏、肾上腺、小肠、胆囊、肺、双侧卵巢和子宫有广泛的淀粉样蛋白沉积。通过质谱法确认淀粉样蛋白类型为ALECT2。肾脏和肝脏中的淀粉样蛋白沉积模式是ALECT2所描述的典型模式。此外,在脾脏、肾上腺、小肠、胆囊、肺、卵巢和子宫中观察到一种独特的淀粉样蛋白沉积模式。ALECT2中的淀粉样蛋白沉积模式与淀粉样蛋白A和淀粉样轻链不同,需要识别以避免误诊为淀粉样轻链或淀粉样蛋白A淀粉样变性。识别后,通过质谱法和/或免疫组织化学染色进行准确诊断对于指导治疗和避免毒性治疗至关重要。