Leukocyte chemotactic factor 2 (LECT2)-associated renal amyloidosis.

摘要

In substantiation of a recent account of a patient whose renal amyloid deposits were formed from leukocyte chemotactic factor 2 (LECT2), we report 10 additional cases in adults ranging in age from 58 to 84 years who exhibited varying degrees of renal functional impairment and proteinuria. Analyses by tandem mass spectrometry of amyloid extracted from sections cut from formalin-fixed, paraffin-embedded blocks revealed, after trypsin digestion, peptides corresponding to the carboxy-terminal portion of the LECT2 molecule; further, the deposits were immunostained by an anti-human LECT2 monoclonal antibody. In four samples analyzed at the molecular level, no mutations in the LECT2-encoding exons were revealed. Based on our studies, we posit that LECT2-associated amyloidosis (ALECT2) represents a unique and perhaps not uncommon disease, the pathogenesis, extent, and prognosis of which remain unknown.