Introduction:
Amyloidosis occurs due to abnormal accumulation of misfolded protein in the parenchyma of organs, often resulting in organ failure. More than 30 different types of amyloidosis are now described in humans. Leukocyte chemotactic factor 2 associated amyloidosis (ALECT2) is one of the most recently described forms, which most commonly affects the kidney and liver. ALECT2 represent an underrecognized etiology of chronic kidney disease with reported bias towards certain ethnic groups.
Methods:
The database from Dr Lal Path Labs, National reference laboratory, Delhi was searched for all cases of amyloidosis diagnosed between January 2023-august 2024. All cases were processed for light and immunofluorescence microscopy using routine techniques. The diagnosis of amyloidosis was made based on congophilia and birefringence on Congo red staining. Amyloid typing was done according to kappa and lambda staining on DIF and IHC stains for SAA and LECT2.
Results:
A total of 19 (3.2%) cases of LECT2 amyloidosis were identified during this time period. ALECT2 was the third most common form of amyloidosis in our study population after AL and AA. ALECT2 primarily presented in patients with a mean age of 61.63 years (32-86 years) with a male preponderance (male: female ratio of 2.1:1). Only 4(21%) patients were from Punjab ethnicity. Most cases presented with renal dysfunction (n=13; 68.4%). LECT2 deposition in post transplant kidneys was found in 10.5% (n=2). The amyloid deposits were present in the renal cortical interstitium in all cases. Most cases showed evidence of glomerular as well as vascular deposition. 52.6% (n=10) of the cases had coexistent histologic diagnoses which included diabetes nephropathy, Membranous glomerulopathy, ANCA vasculitis, IgA nephropathy, DPGN.
Conclusions:
This is the largest study on LECT2 associated amyloidosis in Indian subcontinent. It most commonly presents with slow progressive renal failure and is an underrecognized etiology of chronic kidney disease which usually presents with clinical symptoms of the associated/coexistent disease. Currently, there is no specific treatment for ALECT2 but it is essential to identify this type of amyloidosis so as to prevent mistreatment of the disease with the chemotherapeutic agents that are commonly used for treatment of AL amyloidosis.
I have no potential conflict of interest to disclose.
I did not use generative AI and AI-assisted technologies in the writing process.