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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 2007 Dec;20(4):637-64. doi: 10.1016/j.beha.2007.08.001. PMID: 18070711
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, Noel P
J Clin Oncol 1993 May;11(5):914-20. doi: 10.1200/JCO.1993.11.5.914. PMID: 8487054

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
Kelly JJ
Rev Neurol Dis 2006 Fall;3(4):173-81. PMID: 17224900
Gertz MA, Merlini G, Treon SP
Hematology Am Soc Hematol Educ Program 2004:257-82. doi: 10.1182/asheducation-2004.1.257. PMID: 15561687

Therapy

Nobile-Orazio E
Handb Clin Neurol 2013;115:443-59. doi: 10.1016/B978-0-444-52902-2.00025-4. PMID: 23931795
Sher T, Hayman SR, Gertz MA
Clin Adv Hematol Oncol 2012 Oct;10(10):644-51. PMID: 23187770
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
Kyle RA, Rajkumar SV
Best Pract Res Clin Haematol 2007 Dec;20(4):637-64. doi: 10.1016/j.beha.2007.08.001. PMID: 18070711
Comenzo RL
Curr Treat Options Oncol 2000 Apr;1(1):83-9. doi: 10.1007/s11864-000-0018-9. PMID: 12057064

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
Kyle RA, Rajkumar SV
Best Pract Res Clin Haematol 2007 Dec;20(4):637-64. doi: 10.1016/j.beha.2007.08.001. PMID: 18070711
Gertz MA, Merlini G, Treon SP
Hematology Am Soc Hematol Educ Program 2004:257-82. doi: 10.1182/asheducation-2004.1.257. PMID: 15561687
Berk JL, Keane J, Seldin DC, Sanchorawala V, Koyama J, Dember LM, Falk RH
Chest 2003 Sep;124(3):969-77. doi: 10.1378/chest.124.3.969. PMID: 12970025

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
Kumar SK, Gertz MA, Lacy MQ, Dingli D, Hayman SR, Buadi FK, Short-Detweiler K, Zeldenrust SR, Leung N, Greipp PR, Lust JA, Russell SJ, Kyle RA, Rajkumar SV, Dispenzieri A
Mayo Clin Proc 2011 Jan;86(1):12-8. doi: 10.4065/mcp.2010.0480. PMID: 21193650Free PMC Article
Kumar S, Dispenzieri A, Lacy MQ, Hayman SR, Leung N, Zeldenrust SR, Buadi FK, Kyle RA, Rajkumar SV, Gertz MA
Mayo Clin Proc 2008 Mar;83(3):297-303. doi: 10.4065/83.3.297. PMID: 18315995
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

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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