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Abstract: FR-PO1033

Leukocyte Chemotactic Factor 2 Amyloidosis in an Egyptian Immigrant

Session Information

Category: CKD (Non-Dialysis)

  • 2303 CKD (Non-Dialysis): Mechanisms

Authors

  • Kirby, Madeline, MedStar Georgetown University Hospital Nephrology Services, Washington, District of Columbia, United States
  • Greenberg, Keiko I., MedStar Georgetown University Hospital Nephrology Services, Washington, District of Columbia, United States
  • Kwon, Donghyang, Medstar Georgetown University Hospital Department of Pathology, Washington, District of Columbia, United States
Introduction

Amyloidosis results from the accumulation of misfolded protein in one or more organs. Over 30 amyloid proteins have been discovered1. Identification of the pathogenic amyloid protein is crucial for the management of amyloidosis. Here we present a case of an unexpected cause of amyloidosis.

Case Description

A 69-year-old Egyptian man was referred for evaluation for chronic kidney disease. His history included well-controlled diabetes and hypertension. His creatinine was 1.7 mg/dL, having increased steadily from 0.96 mg/dL five years prior. Urinalysis was bland and urine protein to creatinine ratio was 0.1 g/g. Serologic evaluation was unremarkable except for a monoclonal spike seen on urine protein electrophoresis. He underwent a kidney biopsy to evaluate for monoclonal gammopathy of renal significance. It showed abundant amyloid deposition in the interstitium; amyloid was also seen in the mesangium and glomerular capillary walls. Mass spectrometry analysis at the Mayo Clinic Laboratories identified the amyloid protein not as an immunoglobulin but as leukocyte chemotactic factor 2 (LECT-2). He does not appear to have LECT-2 amyloidosis (ALECT-2) involvement of other organs. He continues to be managed supportively with an ACE-inhibitor and a SGLT-2 inhibitor. Despite these measures, his creatinine continues to increase by 0.2-0.3mg/dL per year.

Discussion

ALECT-2 was first described in 2008 in an individual with nephrotic syndrome2. Nephrotic syndrome has only been found in a minority of cases of ALECT-2 in subsequent series – individuals typically have a bland urine sediment and minimal proteinuria. ALECT-2 commonly affects the kidneys and liver; cardiac involvement appears rare. Its prevalence appears to vary significantly among ethnic groups. In the United States, it has been found most commonly in Hispanic populations, particularly in those of Mexican descent2. ALECT-2 is also common in Egypt; in an Egyptian case series, it was the second most common type of renal amyloidosis, accounting for 31% of cases3. It is unclear why it is more common in certain populations, although a single nucleotide polymorphism of the LECT2 gene has been found in individuals of Mexican ancestry. Currently, there is no specific treatment for ALECT-2. ALECT-2 is likely underdiagnosed due to the frequent lack of proteinuria. An individual’s ancestry may provide a clue toward the diagnosis.