title: [Amyloid light chain amyloidosis].
abstract: Primary amyloid light chain (AL) amyloidosis is the most common and most aggressive form of systemic amyloidosis. In AL amyloidosis, the products of free light chains (FLCs) of monoclonal amyloidogenic plasma cells deposit in the heart, kidneys, liver, gastrointestinal tract, autonomic nerve systems, and soft tissues, consequently leading to progressive disability and organ failure. Tissue biopsy (mainly bone marrow and subcutaneous fat aspirate) staining with Congo red to demonstrate amyloid deposits is required for diagnosis. Autologous stem cell transplant is the preferred treatment method; however, only 25% of patients are eligible. Non-transplant candidates can be offered melphalan-dexamethasone or clinical trials of new agents (thalidomide, lenalidomide, and bortezomib), which have been shown to improve survival. N-terminal pro-brain natriuretic peptide (>1800 ng/l), cardiac troponin T (>0.025 ng/ml), and dFLC (>180 mg/l) are known poor prognostic factors. Late diagnosis remains a major obstacle for initiating effective therapy while organ dysfunction is still recoverable.
authors.affiliation: Department of Hematology, Japanese Red Cross Medical Center.
authors.forename: Kenshi
authors.initials: K
authors.lastname: Suzuki
authors.forename: Toshiko
authors.initials: T
authors.lastname: Shimizu
mesh_terms: D000682:Amyloid / Q000378:metabolism*; D000686:Amyloidosis / Q000175:diagnosis* / Q000276:immunology / Q000378:metabolism / Q000628:therapy*; D018450:Disease Progression; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007136:Immunoglobulins / Q000276:immunology; D014182:Transplantation, Autologous
chemical_list: D000682:Amyloid; D007136:Immunoglobulins