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AL amyloidosis(AL)

MedGen UID:
75674
Concept ID:
C0268381
Disease or Syndrome
Synonyms: AL; Amyloidosis, systemic; Immunoglobulin-related amyloidosis
SNOMED CT: Amyloid light-chain amyloidosis (23132008); Primary amyloidosis of light chain type (23132008); AL amyloidosis (23132008)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0019438
OMIM®: 254500
Orphanet: ORPHA85443

Definition

A clonal B-cell disorder characterized by the aggregation and deposition of insoluble amyloid fibrils derived from misfolding of monoclonal immunoglobulin light chains. It usually presents as systemic AL amyloidosis with involvement of one or more parenchymal organ(s) and, less frequently, as localized amyloidosis with usually nodular deposits restricted to a single organ and/or system. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAL amyloidosis
Follow this link to review classifications for AL amyloidosis in Orphanet.

Professional guidelines

PubMed

Palladini G, Milani P
Drugs 2023 Feb;83(3):203-216. Epub 2023 Jan 18 doi: 10.1007/s40265-022-01830-z. PMID: 36652193
Khwaja J, D'Sa S, Minnema MC, Kersten MJ, Wechalekar A, Vos JM
Haematologica 2022 Sep 1;107(9):2037-2050. doi: 10.3324/haematol.2022.280953. PMID: 35770530Free PMC Article
Adams D, Ando Y, Beirão JM, Coelho T, Gertz MA, Gillmore JD, Hawkins PN, Lousada I, Suhr OB, Merlini G
J Neurol 2021 Jun;268(6):2109-2122. Epub 2020 Jan 6 doi: 10.1007/s00415-019-09688-0. PMID: 31907599Free PMC Article

Recent clinical studies

Etiology

Palladini G, Merlini G
Blood 2022 May 12;139(19):2918-2930. doi: 10.1182/blood.2020008737. PMID: 34517412
Vaxman I, Gertz M
Acta Haematol 2020;143(4):304-311. Epub 2020 Apr 27 doi: 10.1159/000506617. PMID: 32340017
Martinez-Naharro A, Hawkins PN, Fontana M
Clin Med (Lond) 2018 Apr 1;18(Suppl 2):s30-s35. doi: 10.7861/clinmedicine.18-2-s30. PMID: 29700090Free PMC Article
Falk RH, Alexander KM, Liao R, Dorbala S
J Am Coll Cardiol 2016 Sep 20;68(12):1323-41. doi: 10.1016/j.jacc.2016.06.053. PMID: 27634125
Wechalekar AD, Gillmore JD, Hawkins PN
Lancet 2016 Jun 25;387(10038):2641-2654. Epub 2015 Dec 21 doi: 10.1016/S0140-6736(15)01274-X. PMID: 26719234

Diagnosis

Palladini G, Milani P
Drugs 2023 Feb;83(3):203-216. Epub 2023 Jan 18 doi: 10.1007/s40265-022-01830-z. PMID: 36652193
Vaxman I, Gertz M
Acta Haematol 2020;143(4):304-311. Epub 2020 Apr 27 doi: 10.1159/000506617. PMID: 32340017
Martinez-Naharro A, Hawkins PN, Fontana M
Clin Med (Lond) 2018 Apr 1;18(Suppl 2):s30-s35. doi: 10.7861/clinmedicine.18-2-s30. PMID: 29700090Free PMC Article
Falk RH, Alexander KM, Liao R, Dorbala S
J Am Coll Cardiol 2016 Sep 20;68(12):1323-41. doi: 10.1016/j.jacc.2016.06.053. PMID: 27634125
Wechalekar AD, Gillmore JD, Hawkins PN
Lancet 2016 Jun 25;387(10038):2641-2654. Epub 2015 Dec 21 doi: 10.1016/S0140-6736(15)01274-X. PMID: 26719234

Therapy

Wechalekar AD, Sanchorawala V
Blood 2022 Dec 1;140(22):2317-2322. doi: 10.1182/blood.2021014613. PMID: 35507692
Palladini G, Merlini G
Blood 2022 May 12;139(19):2918-2930. doi: 10.1182/blood.2020008737. PMID: 34517412
Kastritis E, Palladini G, Minnema MC, Wechalekar AD, Jaccard A, Lee HC, Sanchorawala V, Gibbs S, Mollee P, Venner CP, Lu J, Schönland S, Gatt ME, Suzuki K, Kim K, Cibeira MT, Beksac M, Libby E, Valent J, Hungria V, Wong SW, Rosenzweig M, Bumma N, Huart A, Dimopoulos MA, Bhutani D, Waxman AJ, Goodman SA, Zonder JA, Lam S, Song K, Hansen T, Manier S, Roeloffzen W, Jamroziak K, Kwok F, Shimazaki C, Kim JS, Crusoe E, Ahmadi T, Tran N, Qin X, Vasey SY, Tromp B, Schecter JM, Weiss BM, Zhuang SH, Vermeulen J, Merlini G, Comenzo RL; ANDROMEDA Trial Investigators
N Engl J Med 2021 Jul 1;385(1):46-58. doi: 10.1056/NEJMoa2028631. PMID: 34192431
Palladini G, Milani P, Merlini G
Blood 2020 Dec 3;136(23):2620-2627. doi: 10.1182/blood.2020006913. PMID: 33270858
Palladini G, Kastritis E, Maurer MS, Zonder J, Minnema MC, Wechalekar AD, Jaccard A, Lee HC, Bumma N, Kaufman JL, Medvedova E, Kovacsovics T, Rosenzweig M, Sanchorawala V, Qin X, Vasey SY, Weiss BM, Vermeulen J, Merlini G, Comenzo RL
Blood 2020 Jul 2;136(1):71-80. doi: 10.1182/blood.2019004460. PMID: 32244252Free PMC Article

Prognosis

Fotiou D, Theodorakakou F, Kastritis E
Int J Mol Sci 2021 Oct 9;22(20) doi: 10.3390/ijms222010916. PMID: 34681575Free PMC Article
Palladini G, Kastritis E, Maurer MS, Zonder J, Minnema MC, Wechalekar AD, Jaccard A, Lee HC, Bumma N, Kaufman JL, Medvedova E, Kovacsovics T, Rosenzweig M, Sanchorawala V, Qin X, Vasey SY, Weiss BM, Vermeulen J, Merlini G, Comenzo RL
Blood 2020 Jul 2;136(1):71-80. doi: 10.1182/blood.2019004460. PMID: 32244252Free PMC Article
Ryšavá R
Nephrol Dial Transplant 2019 Sep 1;34(9):1460-1466. doi: 10.1093/ndt/gfy291. PMID: 30299492
Siddiqi OK, Ruberg FL
Trends Cardiovasc Med 2018 Jan;28(1):10-21. Epub 2017 Jul 13 doi: 10.1016/j.tcm.2017.07.004. PMID: 28739313Free PMC Article
Milani P, Merlini G
Best Pract Res Clin Haematol 2016 Jun;29(2):241-248. Epub 2016 Aug 23 doi: 10.1016/j.beha.2016.08.013. PMID: 27825470

Clinical prediction guides

Uffing A, Hullekes F, Riella LV, Hogan JJ
Clin J Am Soc Nephrol 2021 Nov;16(11):1730-1742. Epub 2021 Oct 22 doi: 10.2215/CJN.00280121. PMID: 34686531Free PMC Article
Boldrini M, Cappelli F, Chacko L, Restrepo-Cordoba MA, Lopez-Sainz A, Giannoni A, Aimo A, Baggiano A, Martinez-Naharro A, Whelan C, Quarta C, Passino C, Castiglione V, Chubuchnyi V, Spini V, Taddei C, Vergaro G, Petrie A, Ruiz-Guerrero L, Moñivas V, Mingo-Santos S, Mirelis JG, Dominguez F, Gonzalez-Lopez E, Perlini S, Pontone G, Gillmore J, Hawkins PN, Garcia-Pavia P, Emdin M, Fontana M
JACC Cardiovasc Imaging 2020 Apr;13(4):909-920. Epub 2019 Dec 18 doi: 10.1016/j.jcmg.2019.10.011. PMID: 31864973
Siddiqi OK, Ruberg FL
Trends Cardiovasc Med 2018 Jan;28(1):10-21. Epub 2017 Jul 13 doi: 10.1016/j.tcm.2017.07.004. PMID: 28739313Free PMC Article
Gillmore JD, Maurer MS, Falk RH, Merlini G, Damy T, Dispenzieri A, Wechalekar AD, Berk JL, Quarta CC, Grogan M, Lachmann HJ, Bokhari S, Castano A, Dorbala S, Johnson GB, Glaudemans AW, Rezk T, Fontana M, Palladini G, Milani P, Guidalotti PL, Flatman K, Lane T, Vonberg FW, Whelan CJ, Moon JC, Ruberg FL, Miller EJ, Hutt DF, Hazenberg BP, Rapezzi C, Hawkins PN
Circulation 2016 Jun 14;133(24):2404-12. Epub 2016 Apr 22 doi: 10.1161/CIRCULATIONAHA.116.021612. PMID: 27143678
Perugini E, Guidalotti PL, Salvi F, Cooke RM, Pettinato C, Riva L, Leone O, Farsad M, Ciliberti P, Bacchi-Reggiani L, Fallani F, Branzi A, Rapezzi C
J Am Coll Cardiol 2005 Sep 20;46(6):1076-84. doi: 10.1016/j.jacc.2005.05.073. PMID: 16168294

Recent systematic reviews

Kumar N, Zhang NJ, Cherepanov D, Romanus D, Hughes M, Faller DV
Orphanet J Rare Dis 2022 Jul 19;17(1):278. doi: 10.1186/s13023-022-02414-6. PMID: 35854312Free PMC Article
Lee C, Lam A, Kangappaden T, Olver P, Kane S, Tran D, Ammann E
J Comp Eff Res 2022 Apr;11(6):451-472. Epub 2022 Feb 21 doi: 10.2217/cer-2021-0261. PMID: 35188424
Ehsan H, Rafae A, Masood A, Wahab A, Sana MK, Ansar I, Neupane K, Umar A, Ehsan A, Hashmi H
Clin Lymphoma Myeloma Leuk 2022 May;22(5):e285-e292. Epub 2021 Nov 1 doi: 10.1016/j.clml.2021.10.014. PMID: 34879994
Bønløkke ST, Ommen HB, Hvas AM
Semin Thromb Hemost 2021 Jul;47(5):569-580. Epub 2021 May 31 doi: 10.1055/s-0041-1725099. PMID: 34058766
Gertz MA, Dispenzieri A
JAMA 2020 Jul 7;324(1):79-89. doi: 10.1001/jama.2020.5493. PMID: 32633805

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