Abstract: TH-PO945
Leukocyte Chemotactic Factor 2 Amyloidosis (ALECT 2) in a Young Female
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
- 1202 Glomerular Diseases: Immunology and Inflammation
Authors
- Kanu, Obiajulu, UNC Hospitals, Nephrology, Chapel Hill, North Carolina, United States
- Khalid, Sheikh Bilal, UNC at Chapel HIll, Chapel Hill, North Carolina, United States
- Howell, David, Duke University and Durham VA Hospitals, Durham, North Carolina, United States
- Jain, Koyal, University of North Carolina Kidney Center, Chapel Hill, North Carolina, United States
Introduction
ALECT2, initially described in 2008 is now the third most common cause of renal amyloidosis. Patients typically present in the seventh decade of life. We describe a unique case of ALECT2 presenting in a young female after a previous biopsy diagnosis of IgA Nephropathy.
Case Description
A 46-year-old Hispanic female with CKD 3 due to IgA nephropathy was evaluated for rising creatinine from 1.7 to 2.7 mg/dL and proteinuria (Urine protein/creatinine 3.5 g/g). She was on losartan, triamterene-hydrochlorothiazide. Repeat kidney biopsy showed moderate to severe interstitial fibrosis and tubular atrophy with 70% globally sclerotic glomeruli on light microscopy. Immunohistochemistry was positive for Ig A and equivalent lambda and kappa stains. Electron microscopy revealed abundant fibrillary deposits and a Congo red stain for amyloid was strongly positive. Liquid chromatography mass spectrometry analysis was consistent with ALECT2 amyloidosis. She was referred to an Oncologist and placed on doxycycline based on evidence of amyloid resorption from studies of other amyloid subtypes. Her creatinine improved (1.7-2mg/dl) although with persistently elevated proteinuria at 3.7g/day.
Discussion
Most cases of ALECT2 described are in older population and it is unclear if age at diagnosis affects prognosis. There is yet no specific treatment for ALECT2. For patients who progress to ESRD, renal transplantation has been shown to be a good therapeutic option. So far, very few studies have described patients with IgA nephropathy and ALECT2. We describe an uncommon case in a young patient, and it highlights the need for thorough re-evaluation of patients with acutely worsening renal function or proteinuria, in the setting of known renal disease. A repeat biopsy is often required in such cases as a different pathology is a possibility.