University of Illinois at Chicago Chicago, IL, United States
Rohit Agrawal, MBBS1, Elie Ghoulam, MD, MS1, Sarang Thaker, MD, MS2, Grace Guzman, MD1, Christine Chan, MD1 1University of Illinois at Chicago, Chicago, IL; 2University of Illinois at Chicago College of Medicine, Chicago, IL
Introduction: Hepatic amyloidosis results from the extracellular deposition of amyloid proteins which due to its three-dimensional ß-lamina structure disrupts the normal tissue architecture and function. Liver involvement is commonly seen in many forms of amyloidosis with variable presentations. More recently a newer protein called Leukocyte cell derived chemotaxin 2 (ALECT-2) is being described in cases of renal amyloidosis. We present a rare case of hepatic amyloidosis due to ALECT-2.
Case Description/Methods: A 78-year-old Hispanic female was referred to our liver clinic for evaluation of asymptomatic elevations of liver chemistries. Her medical history was significant for DM, HTN, chronic kidney disease (CKD) stage 4, hypothyroidism, idiopathic adrenal insufficiency, and bradycardia requiring a pacemaker placement. Her liver function tests were noted to be elevated for around 2 years with peak levels of ALK 165 IU/lt, AST 50 IU/lt and ALT 48 IU/lt. Liver synthetic functions were preserved. Cross-sectional imaging of liver was unremarkable including hepatitis and autoimmune panel along with tests for autosomal recessive disorders. Given persistently elevated liver chemistries, she underwent liver biopsy which revealed eosinophilic deposits on H&E stain (Figure 1A). Congo red stain revealed moderate portal, periportal and sinusoidal amyloid deposits (Figure 1B). Mass spectrometry revealed ALECT-2 amyloid protein deposition. She had a fibrosis stage II/IV. This was the likely cause for her CKD and adrenal insufficiency as well.
Discussion: ALECT-2 protein was first isolated in cases of renal amyloidosis in a cohort of Hispanic patients. Since then, there has been only one case report of hepatic amyloidosis due to ALECT-2 that presented with recurrent variceal bleed. ALECT-2 deposits in liver, spleen, kidney, lungs and adrenal glands. It is expressed in hepatocytes, but little is known about its function. It is hypothesized that it may play a role in affecting tumor suppressor gene expression against hepatocellular carcinoma. Like all amyloidosis, the patients may be asymptomatic or may have abnormal liver chemistries as seen in our patient or may present with cirrhosis and its complications. Biopsy is needed to confirm the diagnosis; however, spectrometry is needed to identify ALECT-2 protein in liver. Little is known regarding the natural course and progression of this type of amyloidosis and that there is no known treatment for this other than supportive management.
Figure: Figure: 1A: H&E stain showing extracellular amorphous, eosinophilic deposits; 1B: Moderate portal, periportal and sinusoidal amyloid deposits on Congo red stain.
Rohit Agrawal indicated no relevant financial relationships.
Elie Ghoulam indicated no relevant financial relationships.
Sarang Thaker indicated no relevant financial relationships.
Grace Guzman indicated no relevant financial relationships.
Christine Chan indicated no relevant financial relationships.
Rohit Agrawal, MBBS1, Elie Ghoulam, MD, MS1, Sarang Thaker, MD, MS2, Grace Guzman, MD1, Christine Chan, MD1. P2923 - Leukocyte Cell Derived Chemotaxin 2 Amyloid Protein, a New Hepatic Pathogen, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.