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Monoclonal light chain cardiac amyloidosis

MedGen UID:
1613161
Concept ID:
C4531197
Disease or Syndrome
HPO: HP:0031326

Definition

A type of cardiac amyloidosis related to deposition of an immunoglobulin light chain. The current gold standard of amyloid typing is to determine the precursor protein using laser microdissection mass spectrometry. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMonoclonal light chain cardiac amyloidosis

Professional guidelines

PubMed

Gertz MA
Am J Hematol 2022 Jun 1;97(6):818-829. Epub 2022 Apr 25 doi: 10.1002/ajh.26569. PMID: 35429180
Stern LK, Patel J
Methodist Debakey Cardiovasc J 2022;18(2):59-72. Epub 2022 Mar 14 doi: 10.14797/mdcvj.1050. PMID: 35414852Free PMC Article
Donnelly JP, Hanna M
Cleve Clin J Med 2017 Dec;84(12 Suppl 3):12-26. doi: 10.3949/ccjm.84.s3.02. PMID: 29257735

Recent clinical studies

Etiology

Gertz MA
Am J Hematol 2022 Jun 1;97(6):818-829. Epub 2022 Apr 25 doi: 10.1002/ajh.26569. PMID: 35429180
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
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

Diagnosis

Gertz MA
Am J Hematol 2022 Jun 1;97(6):818-829. Epub 2022 Apr 25 doi: 10.1002/ajh.26569. PMID: 35429180
Stern LK, Patel J
Methodist Debakey Cardiovasc J 2022;18(2):59-72. Epub 2022 Mar 14 doi: 10.14797/mdcvj.1050. PMID: 35414852Free PMC Article
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
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
Donnelly JP, Hanna M
Cleve Clin J Med 2017 Dec;84(12 Suppl 3):12-26. doi: 10.3949/ccjm.84.s3.02. PMID: 29257735

Therapy

Gertz MA
Am J Hematol 2022 Jun 1;97(6):818-829. Epub 2022 Apr 25 doi: 10.1002/ajh.26569. PMID: 35429180
Stern LK, Patel J
Methodist Debakey Cardiovasc J 2022;18(2):59-72. Epub 2022 Mar 14 doi: 10.14797/mdcvj.1050. PMID: 35414852Free PMC Article
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
Griffin JM, Rosenblum H, Maurer MS
Circ Res 2021 May 14;128(10):1554-1575. Epub 2021 May 13 doi: 10.1161/CIRCRESAHA.121.318187. PMID: 33983835Free PMC Article
Palladini G, Milani P, Merlini G
Blood 2020 Dec 3;136(23):2620-2627. doi: 10.1182/blood.2020006913. PMID: 33270858

Prognosis

Gertz MA
Am J Hematol 2022 Jun 1;97(6):818-829. Epub 2022 Apr 25 doi: 10.1002/ajh.26569. PMID: 35429180
Stern LK, Patel J
Methodist Debakey Cardiovasc J 2022;18(2):59-72. Epub 2022 Mar 14 doi: 10.14797/mdcvj.1050. PMID: 35414852Free PMC Article
Gertz MA, Dispenzieri A
JAMA 2020 Jul 7;324(1):79-89. doi: 10.1001/jama.2020.5493. PMID: 32633805
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
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

Clinical prediction guides

Bhutani D, Lentzsch S
Pharmacol Ther 2020 Oct;214:107612. Epub 2020 Jun 17 doi: 10.1016/j.pharmthera.2020.107612. PMID: 32562825
Qiu Y, Zhang CL, Shen KN, Su W, Feng J, Zhang L, Cao XX, Li J
Ann Hematol 2018 Dec;97(12):2465-2470. Epub 2018 Jul 28 doi: 10.1007/s00277-018-3460-0. PMID: 30056579
Mohty D, Damy T, Cosnay P, Echahidi N, Casset-Senon D, Virot P, Jaccard A
Arch Cardiovasc Dis 2013 Oct;106(10):528-40. Epub 2013 Sep 23 doi: 10.1016/j.acvd.2013.06.051. PMID: 24070600
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
Gertz MA, Kyle RA
Semin Arthritis Rheum 1994 Oct;24(2):124-38. doi: 10.1016/s0049-0172(05)80006-x. PMID: 7839154

Recent systematic reviews

Lakhdar S, Buttar C, Nassar M, Ciobanu C, Patel R, Munira MS
Heart Fail Rev 2022 Nov;27(6):2201-2209. Epub 2022 May 20 doi: 10.1007/s10741-022-10252-8. PMID: 35595919
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
Shragai T, Gatt M, Lavie N, Vaxman I, Tadmor T, Rouvio O, Zektser M, Horowitz N, Magen H, Ballan M, Suru C, Luttwak E, Levi S, Ziv-Baran T, Avivi I, Cohen YC
Eur J Haematol 2021 Feb;106(2):184-195. Epub 2020 Nov 9 doi: 10.1111/ejh.13535. PMID: 33090552
Gertz MA, Dispenzieri A
JAMA 2020 Jul 7;324(1):79-89. doi: 10.1001/jama.2020.5493. PMID: 32633805
Brownrigg J, Lorenzini M, Lumley M, Elliott P
ESC Heart Fail 2019 Oct;6(5):1041-1051. Epub 2019 Sep 5 doi: 10.1002/ehf2.12511. PMID: 31487121Free PMC Article

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