Pt with CHF sx, orthostasis, gastroparesis, and peripheral neuropathy without pmh?

Could consider CHF and new DM but also think about amyloidosis.

Why?
Amyloid fibrils deposit in peripheral nerves = neuropathy.
Autonomic nerves = orthostatic/gastroparesis
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Heart = restrictive CMO, voltage mass discrep (LVH but low voltage)

Edema = heart but also kidney

Often AL Amyloid, need bx often can do fat Pad bx or involved organ.

Send for mass spec for final dx.

If ATTR suspected get technetium pyrophosphate scan for uptake in heart

If ATTR can do tafamadis for neuropathy or liver transplant since the amyloid is made there.

If AL = plasma cell disorder

Get BM Bx, spep, upep, flcs.

Tx= CyBorD and if able to, auto transplant with high dose melphalan conditioning.

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Diff from MM as there VRD (with lenalidomide) is standard if no renal failure, CyBorD if not. In AL amyloid CyBorD is upfront and imids (lenalidomide, pomalidomide) while useful in relapse, cardiotoxicity

Coagulopathy with elevated INR AND PTT?

Factor X def due to adsorption to fibrils.

Recall that factor X is in the common pathway so it affects both the intrinsic (PTT) pathway and extrinsic (PT/INR) pathway