AH amyloidosis

Orpha code: 442582OMIM code:

Definition

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
Classification

Disease

Synonyms
Heavy chain amyloidosis
Amyloidoza immunoglobulinowa
Amyloidoza łańcuchów ciężkich
ORPHA code
442582
OMIM code
-
ICD10 code
E85.9
ICD11 code
-

No additional description.

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