Primary (AL) or light chain disease
Plasma cell dyscrasia (clonal proliferation)
12-15% patients with myeloma have AL
Immunoglobulin light chains
12 month survival without treatment
6 month survival with cardiac disease
Incidence is 1 in 100,000 in Western countries
Familial (AF)
Mutations in transthyretin (TTR)
Ile 122 of particular interest