Amyloidosis classification

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Primary amyloidosis
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Lysozyme amyloid related amyloidosis
Leucocyte cell-derived chemotaxin 2 related amyloidosis
Fibrinogen A alpha-chain associated amyloidosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Syed Hassan A. Kazmi BSc, MD [2]Shaghayegh Habibi, M.D.[3]

Overview

Amyloidosis may be classified on the basis of type of amyloidogenic protein and associated clinical syndromes into primary (AL) amyloidosis, secondary (AA) amyloidosis, familial (AF) amyloidosis, transthyretin (ATTRwt) amyloidosis and dialysis-associated (AH) amyloidosis. It can also be classified based on extent of organ system involvement.

Classification

Classification Based on Precursor of Amyloidogenic Protein: [1][2]

Type Abbreviation Amyloidogenic Protein/Fibril Acquired/Inherited Most Common Organ Involvement Associated Conditions
Primary amyloidosis AL Acquired Heart and kidneys
Secondary amyloidosis AA Acquired Kidneys (early), heart and liver (late)
Senile systemic or wild-type amyloidosis ATTRwt/ATTRvar Acquired (ATTRwt) or Hereditary (ATTRvar) Heart and nerves (more common in hereditary type)
β2-microglobulin related amyloidosis AH Acquired or Hereditary Nerves (peripheral and autonomic)
Leucocyte cell–derived chemotaxin 2 related amyloidosis ALect2
  • Leucocyte cell–derived chemotaxin 2
Acquired Kidneys and liver
Fibrinogen A alpha-chain associated amyloidosis AF Hereditary Kidneys and liver
Abnormal Apolipoprotein A-I, AII, and AIV related amyloidosis AApoA-I Hereditary Kidneys, liver and nerves (peripheral)
Lysozyme amyloid related amyloidosis ALys Hereditary Liver and kidneys
Gelsolin related amyloidosis AGel Hereditary Kidneys and nerves (peripheral and cranial)

Classification Based on Organ Involvement:[3][4]

Classification Subtypes Causes Clinical Features
Systemic amyloidosis Primary amyloidosis (AL)
Secondary amyloidosis (AA)
Hereditary amyloidosis
Organ-specific amyloidosis Renal amyloidosis
Cardiac amyloidosis
Hepatic amyloidosis
Amyloid neuropathy
Gastrointestinal amyloidosis

Refrences

  1. Khoor A, Colby TV (February 2017). "Amyloidosis of the Lung". Arch. Pathol. Lab. Med. 141 (2): 247–254. doi:10.5858/arpa.2016-0102-RA. PMID 28134587.
  2. Benson MD, Buxbaum JN, Eisenberg DS, Merlini G, Saraiva M, Sekijima Y, Sipe JD, Westermark P (December 2018). "Amyloid nomenclature 2018: recommendations by the International Society of Amyloidosis (ISA) nomenclature committee". Amyloid. 25 (4): 215–219. doi:10.1080/13506129.2018.1549825. PMID 30614283. Vancouver style error: initials (help)
  3. Wechalekar AD, Gillmore JD, Hawkins PN (June 2016). "Systemic amyloidosis". Lancet. 387 (10038): 2641–2654. doi:10.1016/S0140-6736(15)01274-X. PMID 26719234.
  4. Falk RH, Alexander KM, Liao R, Dorbala S (September 2016). "AL (Light-Chain) Cardiac Amyloidosis: A Review of Diagnosis and Therapy". J. Am. Coll. Cardiol. 68 (12): 1323–41. doi:10.1016/j.jacc.2016.06.053. PMID 27634125.


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