title: Clinical challenges of an oligosecretory plasma cell dyscrasia.
abstract: Light chain deposition disease (LCDD) and immunoglobulin light chain (AL) amyloidosis are uncommon, and heterogeneous clonal plasma cell (PC) proliferative disorders defined by the different biochemical characteristics of the underlying anomalous immunoglobulin. The deposits are usually multisystemic and the two diseases can coexist. The diagnosis is sometimes made difficult by the absence of a detectable paraprotein by routine immunofixation techniques, and the use of serum-free light chain (FLC) immunoassay brought new value in terms of their diagnosis, prognosis and assessment of treatment response. Association of LCDD and AL amyloidosis with multiple myeloma (MM) at the time of diagnosis is common, but further progression to this condition is considered rare. We present a case of a patient diagnosed with systemic LCDD and AL amyloidosis of atypical biochemical characteristics, with no paraprotein detected in immunoelectrophoresis and immunofixation techniques, who progressed to MM in the later course of her disease.
authors: Department of Nephrology, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal, Portugal. luis.arodrigues@hotmail.com
authors: Luís
authors: L
authors: Rodrigues
authors: Marta
authors: M
authors: Neves
authors: Helena
authors: H
authors: Sá
authors: Mário
authors: M
authors: Campos
mesh_terms: D000328:Adult; D000686:Amyloidosis / Q000150:complications / Q000175:diagnosis; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D006973:Hypertension / Q000209:etiology; D007118:Immunoassay / Q000379:methods; D007145:Immunoglobulin kappa-Chains / Q000378:metabolism; D008099:Liver / Q000473:pathology; D010265:Paraproteinemias / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D010530:Peritoneal Dialysis; D051436:Renal Insufficiency, Chronic / Q000209:etiology / Q000628:therapy; D000683:Serum Amyloid P-Component / Q000378:metabolism
chemical_list: D007145:Immunoglobulin kappa-Chains; D000683:Serum Amyloid P-Component