How are the amyloidoses classified

How are the amyloidoses classified?

By the major protein component of the fibril. This has also become the basis for defining the clinical syndromes with certainty. However, routine stains do not identify the major fibril protein, and specific immunohistochemical stains are needed for identification

Classification of Amyloidoses

Clinical CategoriesMajor Protein TypesRelative Frequencies (%)
Systemic AL amyloidosisAL50–80
Systemic AA amyloidosisAA3–10
Age-related (senile) systemic amyloidosisAATR8–10
Organ-specific (localized) amyloidosisAL, ALECT2, and others a3
Dialysis-related amyloidosisAβ M
Heritable amyloidosisATTR and others b5–10

a Other organ-specific (localized): ALECT2, leukocyte cell-derived chemotaxin 2 – hepatic amyloid; Aβ, amyloid β protein – Alzheimer’s disease, Down syndrome; AIAPP, islet amyloid polypeptide – type 2 diabetes, insulinoma; ACal, calcitonin – medullary thyroid cancer; AANF, atrial natriuretic factor – atrial amyloid; AMed, lactadherin – aortic media; ALac, lactoferrin – cornea; others.

b Other hereditary (familial): AApoA, apolipoprotein AI and AII; AGel, gelsolin; AFib, fibrinogen A alpha; ALys, lysozyme; ACys, cystatin C.


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