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Acute renal failure with lambda light chain-derived crystals in a patient with IgD myeloma
Cécile Toly-Ndour1, Julie Peltier, Rémi Piedagnel
1Institut national de la santé et de la recherche médicale, UMR S 938, système immunitaire et maladies conformationnelles, Hôpital St Antoine, Paris, France.
Abstract:
Kidney involvement with immunoglobulin crystals usually relates to a light chain of the kappa type, in MGUS or smoldering myeloma, frequently causing Fanconi's syndrome with progressive renal insufficiency. We report on a case with severe myeloma featuring lambda light chain-derived crystals and acute kidney injury. Histology showed acute tubular necrosis and tubule obstruction with crystals, which were also abundant inside tubule epithelial cells, macrophages and bone marrow plasma cells. The light chain variable domain had a normal overall primary structure but included 11 somatic mutations, 3 of which likely increased the surface hydrophobicity, as observed in previously reported kappa-type crystals.
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Acute kidney injury develops suddenly and can be caused by pre-renal causes (e.g., hypovolemia, shock), intrinsic renal causes (e.g., acute tubular necrosis), or post-renal causes (e.g., urinary obstruction). In contrast, chronic renal failure progresses gradually over time and is often...
