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🌱 來自: amyloidosis

clinical manifestations of amyloidosis

TLDR: HF, leg edema, nephrotic, free light chain, WARN: when dx as plasma cell or low grade lymphoma?

System	Manifestations	Amyloid Types
Renal	Proteinuria or nephrotic syndrome	AL, AA
Cardiac	Restrictive CMP (↓ EF late), thick walls but ↓ QRS amplitude, conduction abnl, AF, syncope	AL, mATTR, wt ATTR
Pulm	Airway obstruction; pleural effusions	AL, AA
GI	Diarrhea, malabsorption, protein loss; ulcers, hemorrhage, obstruction; macroglossia (dysphonia/dysphagia)	All systemic
HSM	Hepatosplenomegaly w/o hepatic dysfnx or cytopenias	All systemic
Neuro	Periph neuropathy w/ painful paresthesia; carpal tunnel; Autonomic neuro → impotence, dysmotility, ↓ BP	mATTR, Aβ2M, AL, localized
Skin	Waxy, nonpruritic papules; periorbital ecchymoses; “pinch purpura” = skin bleeds with minimal trauma	AL
Endo	Deposition with rare hormonal insufficiency	localized
MSK	Arthralgias and arthritis (especially shoulder)	AL, Aβ2M
Heme	Factor X deficiency	AL

• Factor X deficiency: bleeding risk ~~~~ bx at ↣ safe place
• pinch purpura