Surgical Pathology Criteria

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)
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