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

• 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
• ISSN: 1598-6535
• DOI: 10.3343/kjlm.2009.29.5.384

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.