Light chain proximal tubulopathy: Clinical and pathologic characteristics in the modern treatment era

Authors: Stokes MB, Valeri AM, Herlitz L, et al.

Publication: J Am Soc Nephrol 27: 1555-1565, 2016.

Proximal tubular cells contain cytoplasmic monoclonal immunoglobulin in the entity known as light chain proximal tubulopathy (LCPT).  The deposits are usually crystalline, but can form globules or create vacuoles.  This study examines the clinicopathologic characteristics of these two forms of LCPT.  The majority of cases, (87%), were crystalline, kappa-restricted and had acute tubular injury.  One-third of noncrystalline patients had lambda restriction or acute tubular injury.  Antigen retrieval by immunofluorescence was frequently required to demonstrate both crystalline and noncrystalline forms.  Electron microscopy also demonstrated crystals and granules not seen on light microscopy.