Description of the disease * EnglishPolish Pobierz sekcję do PDF Definicja A rare, systemic amyloidosis characterized by the aggregation and deposition of amyloid fibrils composed of monoclonal immunoglobulin heavy-chain fragments, usually produced by a plasma cell neoplasm. Amyloid fibrils deposit in various organs, most commonly in the kidneys. It typically affects older patients and clinical presentation includes signs and symptoms of renal dysfunction, sometimes leading to nephrotic syndrome and end stage renal disease. Cardiac, liver and nerves involvement has also been described. Disease data Klasyfikacja Disease Synonimy Heavy chain amyloidosis Amyloidoza immunoglobulinowa Amyloidoza łańcuchów ciężkich Kod ORPHA 442582 Kod OMIM - Kod ICD10 E85.9 Kod ICD11 - *Soruce Extended description of the disease Pobierz sekcję do PDF No additional description. Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl