Immunoglobulin Light Chains and the Kidney: An Overview

Abstract

Monoclonal immunoglobulins and free immunoglobulin light chains are produced by plasma cells as a result of their clonal expansion in plasma cell dyscrasia. These proteins are pivotal in the development of pathologic and clinical symptoms of plasma cell dyscrasia and renal manifestations are frequently the presenting and leading features of this process. The spectrum of pathology associated with monoclonal light chains includes light-chain cast nephropathy and tissue deposits derived from the monoclonal light chain, ie, amyloid derived from the light chains and nonamyloidotic light chain deposition disease. The main diagnostic features, differential diagnosis, and pathogenesis are briefly reviewed.

A Forgotten Cause of Kidney Injury in Chronic Myelomonocytic Leukemia

“…It is worth mentioning that kidney injury from other cationic macromolecules has been reported in the literature. Cationic light chains have been shown to cause tubular and glomerular injury independent of cast formation17 in patients with multiple myeloma and B-cell lymphoproliferative disorders. Experimental evidence also suggests direct tubular injury by meglumine, a small cationic molecule commonly used in intravenous contrast agents 18.…”

Free immunoglobulin light chain (FLC) promotes murine colitis and colitis-associated colon carcinogenesis by activating the inflammasome

“…Early clinical reports regarded FLC as biomarkers of many inflammatory diseases, such as autoimmune disease, diabetes mellitus and CNS inflammation 42, 43 . Furthermore, elevated FLC depositing in the extracellular matrix often resulted in inflammation and tissue injury; two examples of this phenomenon include amyloid light chain amyloidosis (AL-AM) and light chain deposition disease (LCDD) 44–46 . Recently, it was found that FLC might be involved in the induction of hypersensitivity reaction by activating mast cells and promoting their degranulation via unknown receptors 47, 48 .…”