Abstract: PO1715
Case of Leukocyte Cell-Derived Chemotaxin 2-Associated Renal Amyloidosis
Session Information
- Glomerular Diseases: Fibrosis and Extracellular Matrix
October 22, 2020 | Location: On-Demand
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1201 Glomerular Diseases: Fibrosis and Extracellular Matrix
Authors
- Kamarzarian, Anita, Olive View Medical Center, Sylmar, California, United States
- Mahmood, Masood, Olive View Medical Center, Sylmar, California, United States
- Jafari, Golriz, Olive View Medical Center, Sylmar, California, United States
Introduction
Amyloidosis is a disorder characterized by the abnormal deposition of insoluble protein fibrils in tissues.The most recently described form of amyloidosis is derived from leukocyte cell-derived chemotaxin 2 (LECT2).
Case Description
60 yo with history of HTN and COPD referred for evlauation of CKD. Creatinine of 1.29 (6/2017) and 1.65 (09/2017). urinalysis no protein and 1 RBC. 24 hour urine protein showed 190 mg of protein. Renal Ultrasound was normal and all other serolgic labs were normal. She had a Renal Biopsy that showed Cango red positive amyloid deposits and Mass Spectromety based proteomic analysis showed peptide profile consistent with ALECT-2 type Amyloid deposition.
Discussion
Ever since the first case of ALECT2 was discovered in 2008, several cases have been reported. ALECT2 affects patients mainly of Hispanic origin, especially Mexican Americans. It is less common in African Americans and Caucasians.
The pathogenesis of this disease is related to accumulation of a protein called LECT2 which was first isolated in 1998. LECT2 protein is a multifunctional factor involved in chemotaxis, inflammation, immunomodulation, and the damage/repair proces.
Most patients with ALECT2 present with minimal proteinuria, bland urine sediment and impaired renal function, and the diagnosis of ALECT2 is usually incidental following biopsies for unrelated conditions or uncertain diagnoses .
ALECT2 is a slowly progressive disease likely due to the selective involvement of the interstitium. A full nephrotic syndrome is uncommon in renal ALECT2. Neither the renal function nor the proteinuria correlates with the amyloid load in the renal biopsy.
There is no specific therapy for ALECT2. Transplantation remains the only effective treatment. But there is a high risk of recurrence in view of ongoing synthesis of the abnormal protein by the liver.
In addition to the renal biopsy findings, confirmation of ALECT2 diagnosis requires immunohistochemistry or chemical analysis by tandem mass spectrometry.