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Differences between κ and λ light chain amyloidosis analyzed by a pathologic scoring system.

Huijuan Wu1, Vincenzo L'Imperio2, Mattia Rossi3

  • 1Department of Pathology, School of Basic Medical Sciences, Fudan University, Shanghai, China.

American Journal of Clinical Pathology
|March 18, 2023
PubMed
Summary

Amyloid light chain (AL)-κ amyloidosis shows higher amyloid burden than AL-λ. This difference in amyloid score (AS) at biopsy may suggest a worse prognosis for AL-κ kidney disease.

Keywords:
amyloidosiskappakidneylambdalight chainpathology

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Area of Science:

  • Nephrology
  • Pathology
  • Immunology

Background:

  • Amyloid light chain (AL) amyloidosis involves light chain proteins depositing in organs.
  • AL-κ and AL-λ are subtypes of AL amyloidosis with shared histopathology.
  • Differences in clinical and histological features between AL-κ and AL-λ remain unclear.

Purpose of the Study:

  • To compare clinical manifestations, histologic findings, and clinical significance between AL-κ and AL-λ amyloidosis subtypes.
  • To evaluate the utility of the composite scarring injury score (CSIS) and amyloid score (AS) in differentiating AL-κ and AL-λ.

Main Methods:

  • Retrospective analysis of 94 kidney biopsies from patients with AL amyloidosis.
  • Evaluation of biopsies using CSIS and AS.
  • Comparison of scores and staining characteristics between AL-κ and AL-λ groups.

Main Results:

  • The amyloid score (AS) was significantly higher in AL-κ than in AL-λ.
  • Capillary wall and vascular amyloid components of AS were higher in AL-κ.
  • Periodic acid-Schiff staining for amyloid was markedly higher in AL-κ; CSIS showed no significant difference between subtypes.

Conclusions:

  • AL-κ amyloidosis exhibits a higher amyloid burden compared to AL-λ at the time of kidney biopsy.
  • Higher serum creatinine and AS in AL-κ suggest a potentially worse prognosis.
  • Findings provide a reference for clinical management of AL amyloidosis subtypes.