Asha Thomas
1 , Ram Prasad Elumalai
1 , Barathi Gunabooshanam
2* , Subalakshmi Balasubramanian
2 , Jayakumar Matcha
1 1 Department of Nephrology, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India
2 Department of Pathology, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India
*Corresponding Author: Assistant Professor, Department of Pathology, Sri Ramachandra Institute of Higher Education and Research, Chennai Email gbarathi@sriramachandra.edu.in
Abstract
Monoclonal gammopathies can produce a variety of glomerular, tubular, vascular and interstitial lesions. The spectrums of renal lesions produced by these monoclonal gammopathies include AL/AH amyloidosis, light chain cast nephropathy or myeloma kidney and various proximal tubulopathies. Out of these, proximal tubule centered lesions are much less identified and diagnosed and light chain proximal tubulopathy (LCPT) is one among them. In LCPT the excess free light chains (mostly kappa type by immunofluorescence microscopy) in serum are filtered by the glomeruli and are reabsorbed by proximal tubules causing its damage. These monoclonal light chains when sequestered in the proximal tubules can give rise to crystalline and noncrystalline histological variants. Here we present a rare case of noncrystalline variant of LCPT with lambda light chain restriction who presented with renal insufficiency which on later investigations revealed to be multiple myeloma.
Implication for health policy/practice/research/medical education:
Among the renal lesions of plasma cell dyscrasias, proximal tubule centred lesions are often overlooked and hence routinely missed during microscopic examination of a renal biopsy specimen. Knowledge about these lesions can be rewarding at times as these can lead the primary treating physician to the underlying disease process when clinical symptoms pertaining to it are absent and early treatment of which can influence the prognosis.
Please cite this paper as: Thomas A, Prasad Elumalai R, Gunabooshanam B, Balasubramanian S, Jayakumar Matcha J. Light chain proximal tubulopathy with lambda restriction presenting as acute kidney injury. J Nephropathol. 2021;10(3):e35. DOI: 10.34172/jnp.2021.35.