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Diagnosis, pathogenesis and outcome in leucocyte chemotactic factor-2 (ALECT2) amyloidosis.

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eMediNexus    12 February 2018

A new article published in Nephrology, Dialysis, Transplantation reported the first case series from a European Centre of prevalence, clinical presentation and diagnostic findings in leucocyte chemotactic factor-2 (ALECT2) amyloidosis and disclosed long-term patient and renal outcomes. This was a study of the clinical features, diagnostic investigations and the outcome of all patients with ALECT2 amyloidosis, followed systematically at the UK National Amyloidosis Centre (NAC), between 1994 and 2015. Among all patients referred to the NAC with biopsy-proved renal amyloid, 24 (1.3%) patients were diagnosed with ALECT2 amyloidosis. The findings revealed that the median estimated glomerular filtration rate (GFR) at diagnosis was 33 mL/min/1.73 m2 and median proteinuria was 0.5 g/24 h. Hepatic amyloid was evident in 11/24 cases, but had no association with significant derangement of liver function. While the evidence of cardiac amyloidosis or amyloid neuropathy was absent in all patients. However, during the median follow-up of 4.8 years, four patients died and four progressed to end-stage renal disease. Meanwhile, the mean rate of GFR loss was 4.2 mL/min/year and median estimated renal survival from diagnosis was 8.2 years. Furthermore, serial SAP scans revealed little or no change in total body amyloid burden. Hence, it was inferred that ALECT2 amyloidosis is a relatively benign type of renal amyloid, associated with a slow GFR decline, which is reliably diagnosed on renal histology.

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