- Systemic illness caused by mis-folded proteins depositing in any organ--> disrupting function
- > 28 variants identified to date
- AL most common, then AA
- Highly variable, dependent on organ affected; kidney most common (AA, AL), then heart
- Often painful, symmetric neuropathy affecting distal upper or lower extremities
- Neuropathy is motor and sensory and can progress rapidly
- Neuropathy can also present w/bilateral carpal tunnel; autonomic dysfxn
- Other target organ damage: Renal→ edema, frothy urine, fatigue; Cardiac→ syncope/presyncope from malignant dysrhythmias, refractory chf; Liver, GI tract→ fatigue, malabsorption, diarrhea, constipation
- Soft tissues, tongue
- Often delays in dx
- Hepatomegaly
- Carpal tunnel
- Findings vary widely, based on organ system affected
- Abnormal VS w/autonomic dysfunction
- Elevated JVP, rales, edema if CHF
- Nephropathy w/peripheral edema
- Symmetric peripheral neuropathy
- Biopsy of affected tissue, stained w/congo red
- Creatinine, UA, urine protein/creatiniine
- SPEP, Serum free light chains