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Medicine
May, 2011;90 (3): 212-222.

Localized amyloidosis of the genitourinary tract: report of 5 new cases and review of the literature.

Matthieu Monge, Dominique Chauveau, Carole Cordonnier, Laure-Hélène Noël, Claire Presne, Raïfah Makdassi, Maïté Jauréguy, Caroline Lecaque, Marianne Renou, Jean-Pierre Grünfeld, Gabriel Choukroun
Author Information
  1. Matthieu Monge: From Departments of Nephrology-Dialysis-Transplantation (MM, CP, RM, MJ, CL, MR, GC) and Pathology (CC), CHU Amiens, Amiens; Department of Nephrology (DC), Rangueil Hospital, Toulouse; Departments of Pathology(LHN) and Nephrology (JPG), Necker Hospital, Paris; and ERI-12, INSERM (MM, GC), Jules Verne University, Amiens, France.
PMID: 21512410 DOI: 10.1097/MD.0b013e31821cbdab

Abstract

Primary localized amyloidosis of the genitourinary tract is a rare entity characterized by small pseudotumors localized in the renal pelvis, ureters, or bladder. Amyloid fibrils are derived from immunoglobulin light chains, but no systemic plasma cell proliferation is detected. The clinical and radiologic features mimic urinary tract cancer, and local treatment is indicated. The prognosis is excellent in most cases, although disease recurrence is possible. We report 5 new cases of localized amyloidosis of the urinary tract, with lambda (4/5), or kappa (1/5) chain amyloid protein, involving the bladder (5/5), and the ureter and renal pelvis (1/5), with multiple, bilateral lesions in 1 case. The presenting complaint was painless hematuria in 4 cases. All cases were of primary (AL)-type amyloidosis. All patients underwent extensive investigation, and none presented any signs of generalized amyloidosis. A favorable outcome was observed in every case. We performed a comprehensive review of the literature, and summarize the data.

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MeSH Term

Adolescent
Adult
Aged
Amyloid
Amyloidosis
Female
Female Urogenital Diseases
Hematuria
Humans
Male
Male Urogenital Diseases
Middle Aged
Prognosis
Recurrence
Treatment Outcome

Chemicals

Amyloid
Case Reports Journal Article Review

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Created with Highcharts 10.0.0amyloidosiscaseslocalizedtractgenitourinaryrenalpelvisbladderurinaryreport5new1/5casereviewliteraturePrimaryrareentitycharacterizedsmallpseudotumorsuretersAmyloidfibrilsderivedimmunoglobulinlightchainssystemicplasmacellproliferationdetectedclinicalradiologicfeaturesmimiccancerlocaltreatmentindicatedprognosisexcellentalthoughdiseaserecurrencepossiblelambda4/5kappachainamyloidproteininvolving5/5uretermultiplebilaterallesions1presentingcomplaintpainlesshematuria4primaryAL-typepatientsunderwentextensiveinvestigationnonepresentedsignsgeneralizedfavorableoutcomeobservedeveryperformedcomprehensivesummarizedataLocalizedtract:

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