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Primary localized amyloidosis

MedGen UID:
1842348
•Concept ID:
C5679901
•
Disease or Syndrome
Synonyms: localised AL amyloidosis; Localized AL amyloidosis; localized AL amyloidosis; primary localized 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:0017817
Orphanet: ORPHA314709

Definition

Primary localized amyloidosis is a form of AL amyloidosis caused by the aggregation of insoluble amyloid fibrils derived from misfolded monoclonal immunoglobulin light chains usually produced by a plasma cell tumor and characterized by localized amyloid deposition with clinical manifestations restricted to the organ involved, most frequently urinary tract (bladder), eye, respiratory tract (larynx, lungs), and skin. [from MONDO]

Term Hierarchy

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

Professional guidelines

PubMed

Treatment of Localized Amyloid Light Chain Amyloidosis With External Beam Radiation Therapy.
Hall J, Rubinstein S, Lilly A, Blumberg JM, Chera B
Pract Radiat Oncol 2022 Nov-Dec;12(6):504-510. Epub 2022 Sep 8 doi: 10.1016/j.prro.2022.03.011. PMID: 36088238
Primary Localized Cutaneous Amyloidosis: A Systematic Treatment Review.
Weidner T, Illing T, Elsner P
Am J Clin Dermatol 2017 Oct;18(5):629-642. doi: 10.1007/s40257-017-0278-9. PMID: 28342017
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 clinical studies

Etiology

Localized amyloidosis of the urinary tract: case series of nine patients.
Merrimen JL, Alkhudair WK, Gupta R
Urology 2006 May;67(5):904-9. Epub 2006 Apr 25 doi: 10.1016/j.urology.2005.11.029. PMID: 16635518
Amyloidosis of the upper aerodigestive tract.
Pribitkin E, Friedman O, O'Hara B, Cunnane MF, Levi D, Rosen M, Keane WM, Sataloff RT
Laryngoscope 2003 Dec;113(12):2095-101. doi: 10.1097/00005537-200312000-00007. PMID: 14660909
Primary localized amyloidosis of the urinary bladder: a case series of 31 patients.
Tirzaman O, Wahner-Roedler DL, Malek RS, Sebo TJ, Li CY, Kyle RA
Mayo Clin Proc 2000 Dec;75(12):1264-8. doi: 10.4065/75.12.1264. PMID: 11126834
Primary localized amyloidosis of the nose and paranasal sinuses. A case report with immunohistochemical observations and a review of the literature.
Mufarrij AA, Busaba NY, Zaytoun GM, Gallo GR, Feiner HD
Am J Surg Pathol 1990 Apr;14(4):379-83. doi: 10.1097/00000478-199004000-00011. PMID: 1690954
Primary localized amyloidosis of urinary bladder.
Caldamone AA, Elbadawi A, Moshtagi A, Frank IN
Urology 1980 Feb;15(2):174-80. doi: 10.1016/0090-4295(80)90416-1. PMID: 6986707

Diagnosis

Primary Amyloidosis of the Genitourinary Tract.
Al-Obaidy KI, Grignon DJ
Arch Pathol Lab Med 2021 Jun 1;145(6):699-703. doi: 10.5858/arpa.2020-0102-RA. PMID: 32383975
Primary localized amyloidosis of the uterine cervix during pregnancy.
Takimoto Y, Kogiku A, Konda E, Samoto T, Ishihara M, Yamashita T
J Obstet Gynaecol Res 2020 May;46(5):787-790. Epub 2020 Feb 19 doi: 10.1111/jog.14218. PMID: 32077184
Primary localized amyloidosis of the stomach mimicking healing gastric ulcer.
Matsueda K, Kawano S, Okada H
Gastrointest Endosc 2020 Apr;91(4):947-948. Epub 2019 Dec 19 doi: 10.1016/j.gie.2019.12.004. PMID: 31863737
Intensity-modulated radiotherapy for localized nasopharyngeal amyloidosis : Case report and literature review.
Luo M, Peng G, Shi L, Ming X, Li Z, Fei S, Ding Q, Cheng J
Strahlenther Onkol 2016 Dec;192(12):944-950. Epub 2016 Jun 14 doi: 10.1007/s00066-016-0996-6. PMID: 27300370
Localized primary ureteral amyloidosis.
García-Escudero López A, Padilla Nieva J, Infante Riaño R, Martín Bazaco J, Arruza Echevarría A, Moreno Nieto V
Arch Esp Urol 2011 May;64(4):371-5. PMID: 21610282

Therapy

The therapeutic role of lasers in primary localized cutaneous amyloidosis: a systematic review.
Ahramiyanpour N, Akbari Z, Sarasyabi MS, Aflatoonian M, Saki N, Shafie'ei M
Lasers Med Sci 2022 Mar;37(2):799-813. Epub 2021 Oct 6 doi: 10.1007/s10103-021-03429-4. PMID: 34611800
Sclerosing thymoma-like thymic amyloidoma with nephrotic syndrome: a case report.
Kato Y, Okuda M, Fukuda K, Tanaka N, Yoshizawa A, Saika Y, Haruna Y, Kitaguchi S, Nohara R
J Med Case Rep 2017 Sep 7;11(1):216. doi: 10.1186/s13256-017-1370-8. PMID: 28877738Free PMC Article
Localized oral amyloidosis of the palate.
Pentenero M, Davico Bonino L, Tomasini C, Conrotto D, Gandolfo S
Amyloid 2006 Mar;13(1):42-6. doi: 10.1080/13506120500537343. PMID: 16690500
Primary localized amyloidosis of the bladder: experience with dimethyl sulfoxide therapy.
Malek RS, Wahner-Roedler DL, Gertz MA, Kyle RA
J Urol 2002 Sep;168(3):1018-20. doi: 10.1016/S0022-5347(05)64564-8. PMID: 12187212
Primary localized amyloidosis of the bladder: a case of AL (lambda) amyloid protein and combination therapy using dimethyl sulfoxide and cepharanthin.
Nishiyama T, Gejyo F, Katayama Y, Takeda M, Takeda M, Sato S
Urol Int 1992;48(2):228-31. doi: 10.1159/000282340. PMID: 1585522

Prognosis

Localized amyloidosis of the genitourinary tract: report of 5 new cases and review of the literature.
Monge M, Chauveau D, Cordonnier C, Noël LH, Presne C, Makdassi R, Jauréguy M, Lecaque C, Renou M, Grünfeld JP, Choukroun G
Medicine (Baltimore) 2011 May;90(3):212-222. doi: 10.1097/MD.0b013e31821cbdab. PMID: 21512410
Amyloidosis of lacrimal gland.
Prabhakaran VC, Babu K, Mahadevan A, Murthy SR
Indian J Ophthalmol 2009 Nov-Dec;57(6):461-3. doi: 10.4103/0301-4738.57160. PMID: 19861750Free PMC Article
Localized primary amyloidosis of the genitourinary tract: does conservatism help?
Zaman W, Singh V, Kumar B, Mandhani A, Srivastava A, Kumar A, Kapoor R
Urol Int 2004;73(3):280-2. doi: 10.1159/000080843. PMID: 15539852
Primary localized amyloidosis of the urinary bladder. A case report.
Anavi BL, Poriazova EG, Staikova ND, Staikov DS
Folia Med (Plovdiv) 2001;43(3):47-52. PMID: 11930833
Primary localized amyloidosis of the urinary bladder: a case series of 31 patients.
Tirzaman O, Wahner-Roedler DL, Malek RS, Sebo TJ, Li CY, Kyle RA
Mayo Clin Proc 2000 Dec;75(12):1264-8. doi: 10.4065/75.12.1264. PMID: 11126834

Clinical prediction guides

Sclerosing thymoma-like thymic amyloidoma with nephrotic syndrome: a case report.
Kato Y, Okuda M, Fukuda K, Tanaka N, Yoshizawa A, Saika Y, Haruna Y, Kitaguchi S, Nohara R
J Med Case Rep 2017 Sep 7;11(1):216. doi: 10.1186/s13256-017-1370-8. PMID: 28877738Free PMC Article
Primary localized amyloidosis of the nose.
Nakayama T, Otori N, Komori M, Takayanagi H, Moriyama H
Auris Nasus Larynx 2012 Feb;39(1):107-9. Epub 2011 May 14 doi: 10.1016/j.anl.2011.01.022. PMID: 21571465
Localized amyloidosis of the genitourinary tract: report of 5 new cases and review of the literature.
Monge M, Chauveau D, Cordonnier C, Noël LH, Presne C, Makdassi R, Jauréguy M, Lecaque C, Renou M, Grünfeld JP, Choukroun G
Medicine (Baltimore) 2011 May;90(3):212-222. doi: 10.1097/MD.0b013e31821cbdab. PMID: 21512410
Pediatric localized amyloidosis in mesopharynx.
Takahashi M, Matsuda H, Ito K, Ito T, Tsukuda M
Int J Pediatr Otorhinolaryngol 2002 Nov 11;66(2):181-4. doi: 10.1016/s0165-5876(02)00215-x. PMID: 12393254
Primary localized amyloidosis of the nose and paranasal sinuses. A case report with immunohistochemical observations and a review of the literature.
Mufarrij AA, Busaba NY, Zaytoun GM, Gallo GR, Feiner HD
Am J Surg Pathol 1990 Apr;14(4):379-83. doi: 10.1097/00000478-199004000-00011. PMID: 1690954

Recent systematic reviews

The therapeutic role of lasers in primary localized cutaneous amyloidosis: a systematic review.
Ahramiyanpour N, Akbari Z, Sarasyabi MS, Aflatoonian M, Saki N, Shafie'ei M
Lasers Med Sci 2022 Mar;37(2):799-813. Epub 2021 Oct 6 doi: 10.1007/s10103-021-03429-4. PMID: 34611800

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