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Commonest forms of systemic amyloidosis

Commonest forms of systemic amyloidosis
Designation* Parent protein Systemic and/or localized Acquired or hereditary Organs involved
AL Immunoglobulin light chain Systemic or localized Acquired (hereditaryΔ) Heart, kidney, liver, soft tissues, peripheral nervous system (including the autonomic nervous system), and gastrointestinal tract
ATTR Transthyretin Systemic Hereditary Peripheral nervous system (including the autonomic nervous system), heart, eye, kidney, and leptomeninges
Systemic Acquired Heart and ligaments
AA Serum amyloid A protein Systemic Acquired Predominantly kidney, but may involve liver, gastrointestinal tract and occasionally heart, thyroid, and autonomic nervous system
ALECT2 Leukocyte chemotactic factor 2 Systemic Acquired Kidney, liver, spleen, adrenals, and lungs
AApoAI Apolipoprotein AI Systemic Hereditary Heart, liver, kidney, peripheral nervous system, testis, larynx, and skin
AFib Fibrinogen alpha chain Systemic Hereditary Kidney, primarily, with obliterative glomerular involvement
ABeta-2-microglobulin-related amyloidosis Beta-2-microglobulin, wild type Systemic Acquired (hemodialysis related) Musculoskeletal system
Beta-2-microglobulin Systemic Hereditary Autonomic nervous system
* The amyloid fibril protein is designated protein A and followed by a suffix that is an abbreviated form of the precursor protein name. For example, when amyloid (A) fibrils are derived from immunoglobulin light (L) chains, the amyloid fibril protein is AL.
¶ Rare cases of amyloidosis formed by immunoglobulin heavy chains (AH) and by heavy and light chains (AHL) have been reported.
Δ One family with mutation in the constant region of the kappa light chain, with cysteine replacing serine at amino acid residue 131, has been reported.
Reprinted by permission from Macmillan Publishers Ltd: Nature Reviews Disease Primers. Merlini G, Dispenzieri A, Sanchorawala V, et al. Systemic immunoglobulin light chain amyloidosis. Nat Rev Dis Primers 2018; 4:38. Copyright © 2018. https://www.nature.com/nrdp/.
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