Experimental Model of Human Light-Chain-Associated Disease

Abstract

The three types of human light-chain-associated disease—“myeloma kidney” disease (myeloma [cast] nephropathy), light-chain deposition disease, and AL amyloidosis—are characterized by renal and systemic deposition of monoclonal light chains, i.e., Bence Jones proteins, as casts, linear precipitates, or fibrils, respectively [1]. These light-chain deposits ultimately result in the impairment of kidney (and other organ) function and account for much of the morbidity and mortality in patients with these disorders [2]. The fact that, clinically and experimentally [1], tubular casts, basement membrane deposits, amyloid formation, and other physiological aberrations are not an invariant accompaniment of Bence Jones proteinuria or are not necessarily related to the amount of monoclonal light chain synthesized or excreted implies that, in addition to host or exogenous factors (e.g., dehydration, hypercalcemia, sepsis, nephrotoxic antibiotics, etc.), certain light chains are “malignant” while others are “benign” [3].