Table 2 Pathological characteristics of patients with light chain proximal tubulopathy
Case #1 | Case #2 | Case #3 | Case #4 | Case #5 | |
|---|---|---|---|---|---|
PTEC | |||||
LM | Focal fuchsinophilic crystal, diffuse vacuolization and swelling | Focal vacuolization and diffuse swelling | Diffuse vacuolization and swelling | Diffuse vacuolization | Focal swelling |
Ig-LC | κ | κ | λ | λ | λ |
IFa | Focal | Focal | Diffuse | Diffuse | Diffuse |
IHCa | Diffuse | Diffuse | Diffuse | Diffuse | Diffuse |
EM | Crystal (rhomboid), lysosome with EDD | Crystal (needle-like), lysosome with EDD and focal dysmorphism | Increased/large/dysmorphic lysosome with mottled appearance | Increased/large/dysmorphic lysosome with mottled appearance | Increased/large/dysmorphic lysosome with mottled appearance |
ATIb | Present, minimal | Present, minimal | Present, minimal | Present | Present, minimal |
IFTAb | Present, minimal | Present, minimal | Present | Present | Absent |
Tubular proteinaceous material | Present | Present | Absent | Present | Present |
Glomerulus | GS (12.1%) | WNL | GS (12.5%) | GS (18.6%) | WNL |
Blood vessel | Fibro-intimal thickening | WNL | Fibro-intimal thickening | Fibro-intimal thickening | Fibro-intimal thickening |