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Kidney Week

Abstract: PO2214

ALECT 2 and Hepatocellular Carcinoma: An Intriguing Association

Session Information

  • Onco-Nephrology - 2
    October 22, 2020 | Location: On-Demand
    Abstract Time: 10:00 AM - 12:00 PM

Category: Trainee Case Report

  • 1500 Onco-Nephrology

Authors

  • Kottey, Janame J., University of Utah Health, Salt Lake City, Utah, United States
  • Yadav, Niraj K., University of Utah Health, Salt Lake City, Utah, United States
  • Abraham, Josephine, University of Utah Health, Salt Lake City, Utah, United States
Introduction

Amyloidosis derived from leukocyte chemotactic factor 2 (ALECT2) is the third most common type of renal amyloidosis in the United States. The incidence of ALECT2 is highest among Hispanics and there is a predilection for involvement of the kidney and liver with sparing of the heart. We report a case of hepatocellular carcinoma (HCC) in a patient with ALECT2

Case Description

A 69-year-old Hispanic man with chronic kidney disease secondary to ALECT2 and monoclonal gammopathy of undetermined significance was admitted for constipation and distended abdomen. Creatinine on a dmission was 8mg/dL and he was initiated on dialysis. Imaging revealed cirrhosis, ascites and two liver masses involving the portal vein. Alpha fetoprotein was 7123ng/mL and he was diagnosed with multifocal HCC. Evaluation for hepatitis, autoimmune disease and other etiologies for chronic liver disease was negative and cirrhosis was presumed to be secondary to ALECT2. Based on the Barcelona Clinic Liver Cancer staging, he was given a stage D. His functional status precluded any liver directed therapies or systemic chemotherapy and he failed to meet the Milan criteria for liver transplantation. Following a discussion of the prognosis he was transitioned to palliative care

Discussion

ALECT2 is a recently described form of systemic amyloidosis that has quickly emerged as a common and possibly underdiagnosed cause of systemic amyloidosis particularly in patients of Hispanic ancestry. ALECT2 can involve various organs but usually spares the heart and brain. ALECT2 in association with HCC has not been reported to date. Interestingly in one study, expression of LECT2 in human HCC biopsies was significantly reduced and over expression of the gene resulted in inhibition of the tumor in an experimental model. ALECT2 is without an identifiable etiology and targeted therapy and hence, it is imperative not to misdiagnose ALECT2 as AL amyloidosis to avoid harmful chemotherapy