Korean J Lab Med.  2009 Oct;29(5):384-389. 10.3343/kjlm.2009.29.5.384.

A Case of Light Chain Deposition Disease Involving Kidney and Bone Marrow with Microangiopathic Hemolytic Anemia

• Cho YU
• Chi HS
• Park CJ
• Jang S
• Cho YM
• Park JS

• KMID: 1077412
• DOI: http://doi.org/10.3343/kjlm.2009.29.5.384

Abstract

We report a case of light chain deposition disease in a 59-yr-old female showing deposition of monoclonal light chain in the kidney and bone marrow accompanied with a schistocytosis, the morphologic finding of microangiopathic hemolytic anemia. The immunofluorescence examination of the kidney revealed strongly stained kappa-light chain deposits on the glomerular mesangium and capillary wall, tubules, and vessel wall. The electron microscopy demonstrated electron-dense deposits on the glomerular basement membrane and mesangium. Anemia was observed with schistocytosis and Howell-Jolly body in the peripheral blood smears. The immunohistochemical examination of the bone marrow showed the presence of kappa-light chain deposits in scattered plasma cells and thickened vessel wall in the absence of a prominent plasma cell proliferation. Although an immunofixation electrophoresis failed to detect a monoclonal gammopathy, the presence of monoclonal protein could be identified by an abnormal kappa/lambda ratio on the serum free light chain analysis.

Keyword

Light chain deposition disease; Kidney; Bone marrow; Microangiopathic hemolytic anemia

MeSH Terms

Anemia, Hemolytic/complications/*diagnosis
Bone Marrow/*pathology
Female
Glomerulonephritis/complications/*diagnosis/pathology
Humans
Immunoglobulin Light Chains/*analysis
Kidney Glomerulus/*pathology/ultrastructure
Middle Aged
Paraproteinemias/complications/*diagnosis/immunology

Figure

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  Fig. 1. Renal biopsy. (A) Nodular glomerulosclerosis characterized by hypocellular nodules in the mesangium (black arrowheads) (PAS stain, ×400). (B) Microaneurysm (arrow) and double contouring (double arrows) of glomerular basement membrane (PA silver stain, ×1,000). (C) Bright staining of glomerulus (arrow) and tubular basement membrane (double arrows), blood vessel, and interstitium (arrowhead) (Immunofluorescence stain with anti-κ light chain antibody, ×200). (D) Very weak stainability in glomerulus (Immunofluorescence stain with anti-λ light chain antibody, ×400).

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  Fig. 2. Ultrastructure of renal glomeruli. (A) Subendothelial electron-dense deposits along the glomerular basement membrane (arrow) (×3,500). (B) Punctate electron-dense deposits in the mesangium (double arrows) (×14,000).

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  Fig. 3. Peripheral blood smear and bone marrow aspirates. (A) Schistocytes and Howell-Jolly body in the peripheral blood smear (Wright stain ×1,000). (B) Plasmacytosis in the bone marrow aspiration (Wright stain, ×1,000).

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  Fig. 4. Bone marrow biopsy. (A) Thickening of vessel wall and plasmacytosis (H&E stain, ×200). (B) Positive reaction in the vessel wall and plasma cells with anti-κ light chain antibody (Immunohistochemcal stain, ×400). (C) Negative reaction with anti-λ light chain antibody (Immunohistochemical stain, ×400).

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