Kidney Week

Abstract: TH-PO940

The Triple Whammy: An Unexpected Case of Leukocyte Chemotactic Factor 2 Amyloidosis (ALECT2) with Diabetic and Hypertensive Nephropathy

Session Information

• Glomerular Trainee Case Reports
  November 07, 2019 | Location: Exhibit Hall, Walter E. Washington Convention Center
  Abstract Time: 10:00 AM - 12:00 PM

Category: Trainee Case Report

• 1201 Glomerular Diseases: Fibrosis and Extracellular Matrix

Authors

• Nandani Tarun, Tanvi, University of Missouri - Columbia, Columbia, Missouri, United States

Tanvi Nandani Tarun,

• Yerram, Preethi, University of Missouri, Columbia, Missouri, United States

Preethi Yerram,

• Alexander, Mariam P., Mayo Clinic, Rochester, Minnesota, United States

Mariam P. Alexander,

Introduction

Amyloidosis is a disorder resulting from abnormal deposition of misfolded beta-sheet fibrils. ALECT2 is a recently identified entity with high incidence in certain ethnic populations.

Case Description

A 56 year old Hispanic female with history of Diabetes Mellitus (not on therapy for a few years), presented with lower extremity edema and elevated blood pressure. Patient was noted to have nephrotic range proteinuria of >4 gm/day, and elevated Creatinine of 3.6 mg/dl. Serological work up for glomerulonephritis was negative. We proceeded with kidney biopsy given lack of proper explanation for her presentation. Biopsy showed evidence of amyloid deposition, but in the context of negative immunofluorescence study, and patient’s ethnicity, LECT2 type amyloid deposition was suspected, which was further identified by Liquid Chromatography (LC) with tandem Mass Spectrometry (MS). Renal biopsy also showed changes consistent with diabetic and hypertensive nephropathy.

Discussion

ALECT2 mostly involves the liver and kidneys. Renal involvement usually presents with slow, progressive renal failure, bland urine sediment, and variable proteinuria. Prognosis of ALECT2 is better than other forms of Amyloidosis, with 1/3^rd of patients progressing to ESRD. Renal transplant is an option, but the disease can recur in the transplanted kidney.Our case report demonstrates the need to maintain a high degree of suspicion for ALECT2 in patients of certain ethnicities such as Hispanics, presenting with renal impairment, bland urine, and variable proteinuria. In addition, it is very important to accurately identify the amyloid deposits by LC/MS on the renal biopsy to prevent misdiagnosing ALECT2 as AL amyloidosis, and avoid potentially harmful investigations/therapy.