A case of light chain deposition disease involving kidney and bone marrow with microangiopathic hemolytic anemia

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 reveal...

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Bibliographic Details
Published in:Taehan Chindan Kŏmsa Ŭihakhoe chi 2009-10, Vol.29 (5), p.384-389
Main Authors: Cho, Young Uk, Chi, Hyun Sook, Park, Chan Jeoung, Jang, Seongsoo, Cho, Yong Mee, Park, Jung Sik
Format: Article
Language:Korean
Subjects:
Anemia, Hemolytic - complications
Anemia, Hemolytic - diagnosis
Bone Marrow - pathology
Female
Glomerulonephritis - complications
Glomerulonephritis - diagnosis
Glomerulonephritis - pathology
Humans
Immunoglobulin Light Chains - analysis
Kidney Glomerulus - pathology
Kidney Glomerulus - ultrastructure
Middle Aged
Paraproteinemias - complications
Paraproteinemias - diagnosis
Paraproteinemias - immunology
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Summary: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.
ISSN:1598-6535
DOI:10.3343/kjlm.2009.29.5.384