ALECT2 Amyloidosis: An Underrecognized Cause of CKD in Hispanic Patients

Introduction: Leukocyte chemotactic factor 2 (LECT2) amyloidosis (ALECT2) is a recently recognized form of amyloidosis, most commonly affecting the kidneys and liver. It is increasingly identified among individuals of Hispanic descent. Unlike AL or AA amyloidosis, ALECT2 typically presents with minimal proteinuria, bland urinary sediment, and slow progression of kidney dysfunction, often leading to delayed diagnosis. Case Description: A 72-year-old Hispanic male with hypertension, type 2 diabetes, and hyperlipidemia was first evaluated in clinic in 2019 with a serum creatinine of 1.88 mg/dL and GFR of 37 mL/min/1.73m. Comorbid conditions were well controlled. He was lost to follow-up until February 2020, when his creatinine rose to 2.09 mg/dL and urine protein-to-creatinine ratio was 0.13 g/g. Serologic workup was unremarkable except for elevated anti-PR3 (6.7 U/mL). Biopsy was recommended but not performed due to loss of follow-up. In January 2025, he returned with creatinine 4.78 mg/dL and GFR 12 mL/min/1.73m. Renal biopsy revealed Congo red-positive deposits with apple-green birefringence. Immunohistochemistry showed equal kappa/lambda staining, negative AA, and focal positivity for LECT2 in glomeruli, interstitium, and vessels, confirming ALECT2 amyloidosis. Due to the progression of the condition the patient started Renal replacement therapy. Discussion: This case highlights the diagnostic challenge posed by ALECT2 due to its indolent course and nonspecific presentation. The condition should be considered in unexplained CKD in high-risk populations, especially Hispanic patients. Early biopsy with appropriate immunohistochemical staining is essential for diagnosis. As no disease-specific therapies exist, management remains supportive, with renal replacement therapy considered in advanced stages.

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Journal
Journal of the American Society of Nephrology
Published
2025-10-01
DOI
https://doi.org/10.1681/asn.20253hm53weq
Primary Topic
Amyloidosis: Diagnosis, Treatment, Outcomes
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article
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article

ALECT2 Amyloidosis: An Underrecognized Cause of CKD in Hispanic Patients

Julio Paolo Zavala, Ashwin Kumar Senthilrajan, Akshay Gunturu
Journal of the American Society of Nephrology
Amyloidosis: Diagnosis, Treatment, Outcomes
article

ALECT2 Amyloidosis: An Underrecognized Cause of CKD in Hispanic Patients

Julio Paolo Zavala, Ashwin Kumar Senthilrajan, Akshay Gunturu
article en

Abstract

Introduction: Leukocyte chemotactic factor 2 (LECT2) amyloidosis (ALECT2) is a recently recognized form of amyloidosis, most commonly affecting the kidneys and liver. It is increasingly identified among individuals of Hispanic descent. Unlike AL or AA amyloidosis, ALECT2 typically presents with minimal proteinuria, bland urinary sediment, and slow progression of kidney dysfunction, often leading to delayed diagnosis. Case Description: A 72-year-old Hispanic male with hypertension, type 2 diabetes, and hyperlipidemia was first evaluated in clinic in 2019 with a serum creatinine of 1.88 mg/dL and GFR of 37 mL/min/1.73m. Comorbid conditions were well controlled. He was lost to follow-up until February 2020, when his creatinine rose to 2.09 mg/dL and urine protein-to-creatinine ratio was 0.13 g/g. Serologic workup was unremarkable except for elevated anti-PR3 (6.7 U/mL). Biopsy was recommended but not performed due to loss of follow-up. In January 2025, he returned with creatinine 4.78 mg/dL and GFR 12 mL/min/1.73m. Renal biopsy revealed Congo red-positive deposits with apple-green birefringence. Immunohistochemistry showed equal kappa/lambda staining, negative AA, and focal positivity for LECT2 in glomeruli, interstitium, and vessels, confirming ALECT2 amyloidosis. Due to the progression of the condition the patient started Renal replacement therapy. Discussion: This case highlights the diagnostic challenge posed by ALECT2 due to its indolent course and nonspecific presentation. The condition should be considered in unexplained CKD in high-risk populations, especially Hispanic patients. Early biopsy with appropriate immunohistochemical staining is essential for diagnosis. As no disease-specific therapies exist, management remains supportive, with renal replacement therapy considered in advanced stages.

Journal of the American Society of NephrologyVol. 36(10S)
University of the Incarnate Word (US)
Openalex Percentile: Top 66%
Amyloidosis: Diagnosis, Treatment, Outcomes
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ALECT2 Amyloidosis: An Underrecognized Cause of CKD in Hispanic Patients — Julio Paolo Zavala, Ashwin Kumar Senthilrajan, et al. · Journal of the American Society of Nephrology (2025) | TGRS Research Map | TGRS