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Kidney Week

Abstract: SA-PO0784

ALECT2: A Rare Cause of Renal Amyloidosis

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

  • Umer, Waseem, Hamad Medical Corporation, Doha, Qatar
  • Abuhelaiqa, Essa, Hamad Medical Corporation, Doha, Qatar
  • Elamin, Nusiba Hafiz, Hamad Medical Corporation, Doha, Qatar
  • Ibrahem, Mahmoud Suliman Mohammed Salih, Hamad Medical Corporation, Doha, Qatar
  • Murshed, Khaled, Hamad Medical Corporation, Doha, Qatar
Introduction

Leukocyte cell-derived chemotaxin 2 (LECT2)-associated amyloidosis is a recently recognized entity and is characterized by a distinctive clinicopathologic type of amyloid deposition manifested in adults by varying degrees of impaired kidney function and proteinuria.

Case Description

65 years old African lady with past medical history of diabetes mellitus type 2 and hypertension, who was evaluated in our nephrology clinic for chronic kidney disease. Her serum creatinine was 177 micromole per litter and estimated glomerular filtration rate (eGFR) of 26ml/min/1.73m2, along with urine protein to creatinine ratio of 286 milligram per millimole with no hematuria on urinalysis. Her examination was significant for elevated Blood Pressure of 185/81mmgh and mild peripheral edema of +1 rest of the examination was unremarkable.Autoimmune workup, was postive for antinuclear antibody (ANA) with titer of 1:320 nucleolar and homogenous pattern, rest of the workup including antineutrophil cytoplasmic antibodies (ANCA),complement levels (C3 and C4),and anti double stranded DNA antibodies, was unremarkable. Serum protein electrophoresis and immunofixation revealed no evidence of monoclonal gammopathy, and serum free light chains were within normal limits, of note, her liver function was normal. Percutaneous renal biopsy was performed which showed markedly enlarged glomeruli with massive mesangial, subendothelial and interstitial deposition of acellular eosinophilic material which silver stain and PAS negative.However the same arease shows apple green bi-refengent with congo red stain. Immunoflorescence were negative for IgA, IgG, IgM, C1q, C3, fibrinogen,kappa and lambda.Liquid chromatography tandem mass spectrometry (LC MS/MS) was performed on peptides extracted from Congo red-positive, microdissected areas detected a peptide profile consistent with ALECT2 (leukocyte chemotactic factor-2)-type amyloid deposition.This finding confirmed our diagnosis.

Discussion

The incdience of ALECT2 associated renal amyloidoss ranges from 2.5% to 2.7% in United States. It has strong predilection to certain ethnic groups including Hispanics,Middle Eastern,Egyptian,Punjabi,and indivduals of Native American ancestry.There are only a limited number of cases reported in the literature and the condition is often underdiagnosed.Accurate diagnosis through histopatholgy is essential to avoid un-necessary and harmful therapy exposure which are commonly used in other types of Amyloidosis.