Variant ABeta2M amyloidosis
Variant ABeta2M amyloidosis
Definition
A rare form of amyloidosis characterized by accumulation and extensive visceral deposition of anamyloidogenic variant of beta 2 microglobulin leading to progressive gastrointestinal dysfunction, Sjögren syndrome and autonomic neuropathy.
Also known as autosomal dominant beta2-microglobulinic amyloidosis — per MONDO