Emphasising the Distinctive Epithelioid Morphology and Clinical Connotation of Hepatic LECT2-Associated Amyloidosis (ALECT2): A Case Report and Review of the Literature

Abstract

Amyloidosis is a protein deposition disorder caused by pathologic accumulation of fibrils, leading to organ dysfunction. The newest 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 resected hepatocellular carcinoma who presented with acute onset jaundice and abnormal liver function tests. Liver biopsy performed for diagnostic workup revealed hepatic ALECT2 with distinct signet-ring like globular proteinaceous deposits infiltrating hepatic parenchyma, mimicking epithelioid/histiocytic neoplasm. The infiltrative spherular material was positive for Congo red and negative for pan-keratin stains. Amyloid protein analysis by Liquid Chromatography tandem Mass Spectrometry (LC MS/MS) identified a peptide profile indicative of ALECT2. Although, ALECT2 accounts for the second most common cause of hepatic amyloidosis, only limited cases of hepatic ALECT2 are reported in the literature and little is known about patient management, especially the role of transplant as curative option. In summary, hepatic ALECT2 is an emerging disorder with relative high prevalence that deserves morphologic recognition. The report intends to emphasise distinctive morphology for accurate diagnosis and understanding its pathogenesis, clinical significance, and therapeutic strategies.

Keywords

• congo red
• hepatic amyloidosis
• mass spectrometry