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Primary systemic amyloidosis

MedGen UID:
129028
Concept ID:
C0281479
Neoplastic Process
Synonym: Primary amyloidosis
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0017816
Orphanet: ORPHA314701

Definition

A plasma cell neoplasm that secretes an abnormal immunoglobulin, which deposits in various tissues and forms a beta-pleated sheet structure that binds Congo red dye with characteristic birefringence. The sites of involvement include heart, liver, kidney, gut, tongue, nerves, and bone. (WHO, 2001) [from NCI]

Professional guidelines

PubMed

Esplin BL, Gertz MA
Curr Probl Cardiol 2013 Feb;38(2):53-96. doi: 10.1016/j.cpcardiol.2012.11.002. PMID: 23337445
Comenzo RL
Curr Treat Options Oncol 2000 Apr;1(1):83-9. doi: 10.1007/s11864-000-0018-9. PMID: 12057064
Gertz MA, Kyle RA
Semin Arthritis Rheum 1994 Oct;24(2):124-38. doi: 10.1016/s0049-0172(05)80006-x. PMID: 7839154

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Systemic Light Chain Amyloidosis, 2023

Recent clinical studies

Etiology

Fend F, Dogan A, Cook JR
Virchows Arch 2023 Jan;482(1):163-177. Epub 2022 Nov 21 doi: 10.1007/s00428-022-03431-3. PMID: 36414803Free PMC Article
Fealey RD
Handb Clin Neurol 2018;157:777-787. doi: 10.1016/B978-0-444-64074-1.00048-3. PMID: 30459040
Kyle RA, Rajkumar SV
Best Pract Res Clin Haematol 2005;18(4):689-707. doi: 10.1016/j.beha.2005.01.025. PMID: 16026745
Comenzo RL
Curr Treat Options Oncol 2000 Apr;1(1):83-9. doi: 10.1007/s11864-000-0018-9. PMID: 12057064
Kyle RA
Annu Rev Med 1989;40:53-60. doi: 10.1146/annurev.me.40.020189.000413. PMID: 2499246

Diagnosis

Fend F, Dogan A, Cook JR
Virchows Arch 2023 Jan;482(1):163-177. Epub 2022 Nov 21 doi: 10.1007/s00428-022-03431-3. PMID: 36414803Free PMC Article
Zhang Y, Feng L
J Cutan Med Surg 2022 Sep-Oct;26(5):541. Epub 2021 Dec 8 doi: 10.1177/12034754211064314. PMID: 34878924
Thaisetthawatkul P, Dyck PJB
Hematol Oncol Clin North Am 2020 Dec;34(6):1091-1098. Epub 2020 Sep 10 doi: 10.1016/j.hoc.2020.07.004. PMID: 33099426
Kyle RA, Rajkumar SV
Best Pract Res Clin Haematol 2005;18(4):689-707. doi: 10.1016/j.beha.2005.01.025. PMID: 16026745
Elston DM, Theresa D, Bryan MG
Cutis 2003 Apr;71(4):276, 279-80. PMID: 12729090

Therapy

Gertz MA, Leung N, Lacy MQ, Dispenzieri A
Kidney Int 2005 Oct;68(4):1464-71. doi: 10.1111/j.1523-1755.2005.00559.x. PMID: 16164623
Patel AL, Jayaram S, Shaikh WA, Basavaraj A, Patel H, Malaviya AP, Kabra D, Patil A, Bhende N
J Assoc Physicians India 2004 Apr;52:335-7. PMID: 15636344
Gertz MA, Rajkumar SV
Curr Treat Options Oncol 2002 Jun;3(3):261-71. doi: 10.1007/s11864-002-0016-1. PMID: 12057072
Comenzo RL
Curr Treat Options Oncol 2000 Apr;1(1):83-9. doi: 10.1007/s11864-000-0018-9. PMID: 12057064
Pramatarov K, Lazarova A, Mateev G, Popov A
Int J Dermatol 1990 Apr;29(3):211-3. doi: 10.1111/j.1365-4362.1990.tb03804.x. PMID: 2186004

Prognosis

Fend F, Dogan A, Cook JR
Virchows Arch 2023 Jan;482(1):163-177. Epub 2022 Nov 21 doi: 10.1007/s00428-022-03431-3. PMID: 36414803Free PMC Article
Thaisetthawatkul P, Dyck PJB
Hematol Oncol Clin North Am 2020 Dec;34(6):1091-1098. Epub 2020 Sep 10 doi: 10.1016/j.hoc.2020.07.004. PMID: 33099426
Schumacher DL
J Contin Educ Nurs 2004 Jan-Feb;35(1):15-6. doi: 10.3928/0022-0124-20040101-06. PMID: 14870905
Gertz MA, Rajkumar SV
Curr Treat Options Oncol 2002 Jun;3(3):261-71. doi: 10.1007/s11864-002-0016-1. PMID: 12057072
Comenzo RL
Curr Treat Options Oncol 2000 Apr;1(1):83-9. doi: 10.1007/s11864-000-0018-9. PMID: 12057064

Clinical prediction guides

Karasuyama T, Honma Y, Kumamoto K, Shibata M, Watanabe T, Shimajiri S, Abe S, Yamashita T, Harada M
J UOEH 2021;43(2):227-233. doi: 10.7888/juoeh.43.227. PMID: 34092767
Gertz MA, Merlini G, Treon SP
Hematology Am Soc Hematol Educ Program 2004:257-82. doi: 10.1182/asheducation-2004.1.257. PMID: 15561687
Gertz MA, Kyle RA
Semin Arthritis Rheum 1994 Oct;24(2):124-38. doi: 10.1016/s0049-0172(05)80006-x. PMID: 7839154
Gertz MA, Kyle RA
Am J Clin Pathol 1990 Sep;94(3):313-7. PMID: 2118721
Gertz MA, Kyle RA, Greipp PR
Blood 1989 Aug 15;74(3):1108-11. PMID: 2752156

Recent systematic reviews

Mhaskar R, Kumar A, Behera M, Kharfan-Dabaja MA, Djulbegovic B
Biol Blood Marrow Transplant 2009 Aug;15(8):893-902. Epub 2009 Apr 2 doi: 10.1016/j.bbmt.2009.01.022. PMID: 19589478

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Curated

    • NCCN, 2023
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Systemic Light Chain Amyloidosis, 2023

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