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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.
See: This record
Not genetically inherited (Orphanet)
 
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]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPrimary systemic amyloidosis

Professional guidelines

PubMed

Current trends in diagnosis and management of cardiac amyloidosis.
Esplin BL, Gertz MA
Curr Probl Cardiol 2013 Feb;38(2):53-96. doi: 10.1016/j.cpcardiol.2012.11.002. PMID: 23337445
Primary systemic amyloidosis.
Comenzo RL
Curr Treat Options Oncol 2000 Apr;1(1):83-9. doi: 10.1007/s11864-000-0018-9. PMID: 12057064
Amyloidosis: prognosis and treatment.
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

Plasma cell neoplasms and related entities-evolution in diagnosis and classification.
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
Thermoregulation in neuropathies.
Fealey RD
Handb Clin Neurol 2018;157:777-787. doi: 10.1016/B978-0-444-64074-1.00048-3. PMID: 30459040
Pleural effusions in systemic amyloidosis.
Berk JL
Curr Opin Pulm Med 2005 Jul;11(4):324-8. doi: 10.1097/01.mcp.0000162378.35928.37. PMID: 15928500
Primary systemic amyloidosis.
Comenzo RL
Curr Treat Options Oncol 2000 Apr;1(1):83-9. doi: 10.1007/s11864-000-0018-9. PMID: 12057064
Monoclonal gammopathies and the kidney.
Kyle RA
Annu Rev Med 1989;40:53-60. doi: 10.1146/annurev.me.40.020189.000413. PMID: 2499246

Diagnosis

Plasma cell neoplasms and related entities-evolution in diagnosis and classification.
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
Primary Systemic Amyloidosis.
Zhang Y, Feng L
J Cutan Med Surg 2022 Sep-Oct;26(5):541. Epub 2021 Dec 8 doi: 10.1177/12034754211064314. PMID: 34878924
Hepatocyte Growth Factor and Primary Systemic Amyloidosis.
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
Peripheral Nervous System Involvement.
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
Primary systemic amyloidosis.
Gertz MA, Rajkumar SV
Curr Treat Options Oncol 2002 Jun;3(3):261-71. doi: 10.1007/s11864-002-0016-1. PMID: 12057072

Therapy

Primary Systemic Amyloidosis: A Case Report.
Oli SS, Jha A, Karki A, Sapkota S, Adhikari L
JNMA J Nepal Med Assoc 2023 Oct 1;61(266):822-824. doi: 10.31729/jnma.8297. PMID: 38289775Free PMC Article
Neuropathy and monoclonal gammopathy.
Nobile-Orazio E
Handb Clin Neurol 2013;115:443-59. doi: 10.1016/B978-0-444-52902-2.00025-4. PMID: 23931795
Primary systemic amyloidosis.
Gertz MA, Rajkumar SV
Curr Treat Options Oncol 2002 Jun;3(3):261-71. doi: 10.1007/s11864-002-0016-1. PMID: 12057072
Primary systemic amyloidosis.
Comenzo RL
Curr Treat Options Oncol 2000 Apr;1(1):83-9. doi: 10.1007/s11864-000-0018-9. PMID: 12057064
Amyloidosis: prognosis and treatment.
Gertz MA, Kyle RA
Semin Arthritis Rheum 1994 Oct;24(2):124-38. doi: 10.1016/s0049-0172(05)80006-x. PMID: 7839154

Prognosis

Plasma cell neoplasms and related entities-evolution in diagnosis and classification.
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
Hepatocyte Growth Factor and Primary Systemic Amyloidosis.
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
Peripheral Nervous System Involvement.
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
Primary systemic amyloidosis.
Gertz MA, Rajkumar SV
Curr Treat Options Oncol 2002 Jun;3(3):261-71. doi: 10.1007/s11864-002-0016-1. PMID: 12057072
Primary systemic amyloidosis.
Comenzo RL
Curr Treat Options Oncol 2000 Apr;1(1):83-9. doi: 10.1007/s11864-000-0018-9. PMID: 12057064

Clinical prediction guides

Hepatocyte Growth Factor and Primary Systemic Amyloidosis.
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
Amyloidosis and Waldenström's macroglobulinemia.
Gertz MA, Merlini G, Treon SP
Hematology Am Soc Hematol Educ Program 2004:257-82. doi: 10.1182/asheducation-2004.1.257. PMID: 15561687
Primary (AL) hepatic amyloidosis: clinical features and natural history in 98 patients.
Park MA, Mueller PS, Kyle RA, Larson DR, Plevak MF, Gertz MA
Medicine (Baltimore) 2003 Sep;82(5):291-8. doi: 10.1097/01.md.0000091183.93122.c7. PMID: 14530778
Survival in patients with primary systemic amyloidosis and raised serum cardiac troponins.
Dispenzieri A, Kyle RA, Gertz MA, Therneau TM, Miller WL, Chandrasekaran K, McConnell JP, Burritt MF, Jaffe AS
Lancet 2003 May 24;361(9371):1787-9. doi: 10.1016/S0140-6736(03)13396-X. PMID: 12781539
Amyloidosis: prognosis and treatment.
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

Role of high-dose chemotherapy and autologous hematopoietic cell transplantation in primary systemic amyloidosis: a systematic review.
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

    Reviews

    Related information

    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

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