Primary Amyloidosis
Differential Diagnosis
Amyloidoses and Deposition Diseases
| Primary (AL) |
Secondary (AA) |
Familial (AF) |
Hemodialysis Associated (β2) |
Light/Heavy Chain Deposition Disease |
| M component in 80% (usually lambda) |
No M component |
No M component |
No M component |
M component often present (usually kappa) |
| Clonal plasma cells |
No clonal population |
No clonal population |
No clonal population |
Clonal plasma cells may be demonstrable in marrow |
| Clonal light chain in deposits may be demonstrable |
No clonal light chain deposits |
No clonal light chain deposits |
No clonal light chain deposits |
Clonal light/heavy chain deposits may be demonstrable |
| Congo Red positive |
Congo Red positive |
Congo Red positive |
Congo Red negative |
Congo Red negative |
Heavy chain deposition disease is very rare, so the following must be ruled out
Lymphoplasmacytic lymphoma with IgG deposition
SLL/CLL witih IgM deposition
Extranodal marginal zone lymphoma with IgA deposition (IPSID)
