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Primary localized amyloidosis

MedGen UID:
799771
•Concept ID:
CN203787
•
Finding
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
Follow this link to review classifications for Primary localized amyloidosis in Orphanet.

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
European myeloma network recommendations on diagnosis and management of patients with rare plasma cell dyscrasias.
Gavriatopoulou M, Musto P, Caers J, Merlini G, Kastritis E, van de Donk N, Gay F, Hegenbart U, Hajek R, Zweegman S, Bruno B, Straka C, Dimopoulos MA, Einsele H, Boccadoro M, Sonneveld P, Engelhardt M, Terpos E
Leukemia 2018 Sep;32(9):1883-1898. Epub 2018 Jul 23 doi: 10.1038/s41375-018-0209-7. PMID: 30038381
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

Recent clinical studies

Etiology

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 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
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
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 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
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
Localized conjunctival amyloidosis.
Blodi FC, Apple DJ
Am J Ophthalmol 1979 Sep;88(3 Pt 1):346-50. doi: 10.1016/0002-9394(79)90631-7. PMID: 484663

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
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
A case of primary localized amyloidosis in both tonsils.
Eriksen HE
J Laryngol Otol 1970 May;84(5):525-31. doi: 10.1017/s0022215100072182. PMID: 5428061

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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