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首页> 外文期刊>Journal of Clinical and Diagnostic Research >Emphasising the Distinctive Epithelioid Morphology and Clinical Connotation of Hepatic LECT2-Associated Amyloidosis (ALECT2): A Case Report and Review of the Literature
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Emphasising the Distinctive Epithelioid Morphology and Clinical Connotation of Hepatic LECT2-Associated Amyloidosis (ALECT2): A Case Report and Review of the Literature

机译：强调与肝LECT2相关的淀粉样变性病（ALECT2）的独特上皮形态和临床意义：一例病例并文献复习

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摘要

Amyloidosis is a protein deposition disorder caused by pathologic accumulation of fbrils, leading to organ dysfunction. Thenewest protein is Leukocyte Cell-derived Chemotaxin 2 Amyloidosis (ALECT2), which shows ethnic predilection for Hispanics,Middle Eastern, and other minority groups. We report a case of 71-year-old Persian male with history of hepatitis B and resectedhepatocellular carcinoma who presented with acute onset jaundice and abnormal liver function tests. Liver biopsy performedfor diagnostic workup revealed hepatic ALECT2 with distinct signet-ring like globular proteinaceous deposits infltrating hepaticparenchyma, mimicking epithelioid/histiocytic neoplasm. The infltrative spherular material was positive for Congo red and negativefor pan-keratin stains. Amyloid protein analysis by Liquid Chromatography tandem Mass Spectrometry (LC MS/MS) identifed apeptide profle indicative of ALECT2. Although, ALECT2 accounts for the second most common cause of hepatic amyloidosis, onlylimited cases of hepatic ALECT2 are reported in the literature and little is known about patient management, especially the roleof transplant as curative option. In summary, hepatic ALECT2 is an emerging disorder with relative high prevalence that deservesmorphologic recognition. The report intends to emphasise distinctive morphology for accurate diagnosis and understanding itspathogenesis, clinical signifcance, and therapeutic strategies.
机译：淀粉样变性是由原纤维的病理性积累引起的蛋白质沉积障碍，导致器官功能障碍。最新的蛋白质是白细胞衍生的化学趋化因子2淀粉样变性（ALECT2），对西班牙裔，中东和其他少数族裔表现出种族偏爱。我们报告一例71岁的波斯男性，患有乙型肝炎和切除的肝细胞癌，并伴有急性起病性黄疸和异常肝功能检查。为诊断检查而进行的肝活检显示，肝ALECT2具有明显的印戒，像球状蛋白沉积物，浸润肝实质，模仿上皮样/组织细胞性肿瘤。刚果红为阳性球状物质，泛角蛋白染色为阴性。通过液相色谱串联质谱（LC MS / MS）进行的淀粉样蛋白分析可鉴定出表明ALECT2的肽谱。尽管ALECT2是造成肝淀粉样变性的第二大最常见原因，但文献中仅报道了有限的肝ALECT2病例，对患者的治疗知之甚少，尤其是移植作为治疗选择的作用。总之，肝ALECT2是一种新兴疾病，患病率较高，值得形态学上的认可。该报告旨在强调独特的形态，以进行准确的诊断并了解其发病机理，临床意义和治疗策略。

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