A case of renal and splenic LECT 2 amyloidosis: A recently recognized cause of renal and systemic amyloidosis

Saudi J Kidney Dis Transpl. 2020 Mar-Apr;31(2):508-514. doi: 10.4103/1319-2442.284027.

Abstract

Amyloidosis has traditionally been of a few defined varieties, most commonly including light-chain amyloidosis (AL amyloidosis) and secondary amyloidosis due to chronic inflammation (AA amyloidosis). Apolipoprotein A-I/A-II cystatin C, gelsolin, lysozyme, fibrinogen alpha chain, beta 2 microglobulin, and transthyretin familial amyloidosis represent rarer but reported varieties. Ten years ago, the first reports linked leukocyte chemotactic factor 2 (LECT2) amyloidosis as a pathological agent identified as a novel class of amyloid-generating protein. Epidemiology suggested that this was a new cause of amyloidosis that is especially common in Hispanic patients and somewhat common among patients from the Middle East-North Africa (MENA) region. We report a case of splenic and renal LECT 2 amyloidosis in a 62-year- old Hispanic male with diabetes mellitus. After an unremarkable serological workup, LECT 2 amyloidosis was diagnosed on renal biopsy. The case presentation is reviewed as a typical presentation, and the literature is reviewed regarding this newly reported entity, resulting in infiltrative renal amyloidosis and chronic renal disease.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Amyloidosis / diagnosis*
  • Amyloidosis / metabolism
  • Amyloidosis / pathology
  • Amyloidosis / therapy
  • Biomarkers / analysis
  • Biopsy
  • Humans
  • Intercellular Signaling Peptides and Proteins / analysis
  • Kidney / chemistry*
  • Kidney / ultrastructure
  • Male
  • Microscopy, Electron
  • Middle Aged
  • Predictive Value of Tests
  • Renal Insufficiency, Chronic / diagnosis*
  • Renal Insufficiency, Chronic / metabolism
  • Renal Insufficiency, Chronic / pathology
  • Renal Insufficiency, Chronic / therapy
  • Splenic Diseases / diagnosis*
  • Splenic Diseases / metabolism
  • Splenic Diseases / pathology
  • Splenic Diseases / therapy
  • Staining and Labeling
  • Treatment Outcome

Substances

  • Biomarkers
  • Intercellular Signaling Peptides and Proteins
  • LECT2 protein, human