Amyloidoza AH

Kod Orpha: 442582Kod OMIM:

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.

Dane
Klasyfikacja

Choroba

Synonimy
Heavy chain amyloidosis
Amyloidoza immunoglobulinowa
Amyloidoza łańcuchów ciężkich
Kod ORPHA
442582
Kod OMIM
-
Kod ICD10
E85.9
Kod ICD11
5D00.Y

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