Leukocyte chemotactic factor 2 (LECT2)-associated renal amyloidosis: a case series

Am J Kidney Dis. 2010 Dec;56(6):1100-7. doi: 10.1053/j.ajkd.2010.08.013. Epub 2010 Oct 16.


Background: Renal amyloidosis is characterized by the pathologic deposition within glomeruli and/or interstitium of congophilic fibrils, most often composed of either immunoglobulin light chains or serum amyloid A-related protein and, less commonly, mutated forms of apolipoproteins AI or AII, lysozyme, fibrinogen, gelsolin, or transthyretin.

Study design: Case series.

Setting & participants: 10 patients with renal amyloidosis who had an amyloidogenic protein that was not identified using routine immunohistochemistry.

Outcomes: Clinical, pathologic, biochemical, and genetic characteristics.

Measurements: Tandem mass spectrometry was used to analyze fibrils extracted from sections of formalin-fixed paraffin-embedded amyloid-containing kidney biopsy specimen blocks.

Results: Chemical analyses showed peptides corresponding to the carboxy-terminal portion of the leukocyte chemotactic factor 2 (LECT2) molecule. In addition, deposits were immunostained using an anti-human LECT2 monoclonal antibody. Plasma specimens were available from 2 individuals for whom LECT2 concentration in these samples was within the reference range. Additionally, in 4 of the cases analyzed at the molecular level, isolation of genomic DNA and polymerase chain reaction amplification of LECT2-encoding exons showed no mutations. However, all were homozygous for the G allele encoding valine at position 40 in the mature protein, a finding confirmed using restriction enzyme analysis of the polymorphic site.

Limitations: Causality is not addressed.

Conclusions: Based on our studies, we posit that LECT2-associated renal amyloidosis represents a unique and perhaps not uncommon disease, especially in Mexican Americans. The pathogenesis, extent, and prognosis remain to be determined.

Publication types

  • Case Reports
  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Aged, 80 and over
  • Amyloidosis / genetics
  • Amyloidosis / metabolism*
  • Amyloidosis / pathology
  • Biopsy
  • Exons / genetics
  • Female
  • Homozygote
  • Humans
  • Intercellular Signaling Peptides and Proteins / metabolism*
  • Kidney / metabolism
  • Kidney / pathology
  • Kidney Diseases / genetics
  • Kidney Diseases / metabolism*
  • Kidney Diseases / pathology
  • Male
  • Mexican Americans / genetics
  • Middle Aged


  • Intercellular Signaling Peptides and Proteins
  • LECT2 protein, human