open access

Vol 9, No 4 (2018)
Case report
Published online: 2019-03-06
Get Citation

Amyloidosis of the urinary bladder — case report and literature review

Anna Suska12, Dorota Rowczenio3, Janusz Jaszczyński4, Wojciech Szot5, Sarah Goldman-Mazur16, Artur Jurczyszyn16
DOI: 10.5603/Hem.2018.0039
·
Hematologia 2018;9(4):330-335.
Affiliations
  1. Katedra Hematologii, Collegium Medicum, Uniwersytet Jagielloński, Kraków
  2. Oddział Obserwacyjno-Internistyczny, Szpital Uniwersytecki w Krakowie
  3. National Amyloidosis Centre, UCL Division of Medicine, Royal Free Hospital, Londyn, United Kingdom
  4. Klinika Chirurgii Onkologicznej, Centrum Onkologii — Instytut im. Marii Skłodowskiej-Curie, Oddział w Krakowie, Kraków, Poland
  5. Zakład Medycyny Nuklearnej, Krakowski Szpital Specjalistyczny im. Jana Pawła II, Poland
  6. Oddział Kliniczny Hematologii, Szpital Uniwersytecki w Krakowie

open access

Vol 9, No 4 (2018)
CASE REPORTS
Published online: 2019-03-06

Abstract

Amyloidosis is a heterogeneous group of protein misfolding diseases caused by extracellular deposition of abnormal beta-fibrils resistant to proteolysis. The most common type is light-chain amyloidosis (AL amyloidosis) which can be systemic or localized. Localized amyloidosis mostly affects the respiratory airways, genitourinary tract, gastrointestinal system or skin. We present diagnostic and therapeutic approach in a 25-year-old female patient diagnosed with urinary bladder amyloidosis. The main complaint was macroscopic hematuria occurring periodically for 2 years. Abdominal and pelvic computed tomography revealed single 25-mm-thick soft-tissue lesion of the left bladder wall. Histopathological examination of the lesion biopsied during transurethral resection of bladder tumor showed amyloid deposits with strong positive immunostaining for transthyretin, weaker for light chain (AL) and weak for serum amyloid A (AA). Serum protein electrophoresis and immunofixation did not reveal monoclonal protein. X-rays of flat bones presented without lytic lesions. There were no amyloid deposits both in trephine biopsy and subcutaneous fat biopsy. Primary systemic AL amyloidosis was excluded. According to the results of (99m)Tc-DPD scintigraphy and genetic analysis of transthyretin gene (TTR), the ATTRm amyloidosis was also excluded. Consultative histopathological analysis of the bladder biopsy made in the National Amyloidosis Center in London revealed amyloid deposits stained to lambda light chains, confirming the diagnosis of localized AL lambda bladder amyloidosis. The patient was qualified for surgical treatment. Partial resection of the bladder wall with no need for left ureter transplantation was performed. Primary localized bladder amyloidosis is a very rare entity. In the diagnostic approach the most important is the exclusion of primary systemic amyloidosis due to a completely different treatment method.

Abstract

Amyloidosis is a heterogeneous group of protein misfolding diseases caused by extracellular deposition of abnormal beta-fibrils resistant to proteolysis. The most common type is light-chain amyloidosis (AL amyloidosis) which can be systemic or localized. Localized amyloidosis mostly affects the respiratory airways, genitourinary tract, gastrointestinal system or skin. We present diagnostic and therapeutic approach in a 25-year-old female patient diagnosed with urinary bladder amyloidosis. The main complaint was macroscopic hematuria occurring periodically for 2 years. Abdominal and pelvic computed tomography revealed single 25-mm-thick soft-tissue lesion of the left bladder wall. Histopathological examination of the lesion biopsied during transurethral resection of bladder tumor showed amyloid deposits with strong positive immunostaining for transthyretin, weaker for light chain (AL) and weak for serum amyloid A (AA). Serum protein electrophoresis and immunofixation did not reveal monoclonal protein. X-rays of flat bones presented without lytic lesions. There were no amyloid deposits both in trephine biopsy and subcutaneous fat biopsy. Primary systemic AL amyloidosis was excluded. According to the results of (99m)Tc-DPD scintigraphy and genetic analysis of transthyretin gene (TTR), the ATTRm amyloidosis was also excluded. Consultative histopathological analysis of the bladder biopsy made in the National Amyloidosis Center in London revealed amyloid deposits stained to lambda light chains, confirming the diagnosis of localized AL lambda bladder amyloidosis. The patient was qualified for surgical treatment. Partial resection of the bladder wall with no need for left ureter transplantation was performed. Primary localized bladder amyloidosis is a very rare entity. In the diagnostic approach the most important is the exclusion of primary systemic amyloidosis due to a completely different treatment method.
Get Citation

Keywords

AL amyloidosis, urinary bladder, localized amyloidosis

About this article
Title

Amyloidosis of the urinary bladder — case report and literature review

Journal

Hematology in Clinical Practice

Issue

Vol 9, No 4 (2018)

Article type

Case report

Pages

330-335

Published online

2019-03-06

Page views

646

Article views/downloads

741

DOI

10.5603/Hem.2018.0039

Bibliographic record

Hematologia 2018;9(4):330-335.

Keywords

AL amyloidosis
urinary bladder
localized amyloidosis

Authors

Anna Suska
Dorota Rowczenio
Janusz Jaszczyński
Wojciech Szot
Sarah Goldman-Mazur
Artur Jurczyszyn

References (9)
  1. Dmoszyńska A, Usnarska-Zubkiewicz L, Walewski J, et al. Zalecenia Polskiej Grupy Szpiczakowej dotyczące rozpoznawania i leczenia szpiczaka plazmocytowego oraz innych dyskrazji plazmocytowych na rok 2017. Acta Haematol Pol. 2017; 48(2): 55–103.
  2. Puła B, Dębek S, Jamroziak K. Zasady klasyfikacji i nazewnictwa amyloidoz. Hematologia. 2018; 9(3): 167–172.
  3. Suska A, Jurczyszyn A. Fluktuujący zespół Guillaina-Barré w przebiegu systemowej amyloidozy AL — zwiastun progresji? Hematologia. 2018; 9(3): 239–244.
  4. Malek RS, Wahner-Roedler DL, Gertz MA, et al. Primary localized amyloidosis of the bladder: experience with dimethyl sulfoxide therapy. J Urol. 2002; 168(3): 1018–1020.
  5. Merrimen JLO, Alkhudair WK, Gupta R. Localized amyloidosis of the urinary tract: case series of nine patients. Urology. 2006; 67(5): 904–909.
  6. Tirzaman O, Wahner-Roedler DL, Malek RS, et al. Primary localized amyloidosis of the urinary bladder: a case series of 31 patients. Mayo Clin Proc. 2000; 75(12): 1264–1268.
  7. Altwairgi A. Primary amyloidosis of the urinary bladder presenting as painless heamaturia. Int J Health Sci (Qassim). 2011; 5(2): 181–185.
  8. Alsikafi NF, O'Connor RC, Yang XJ, et al. Primary amyloidosis of the bladder treated with partial cystectomy. Can J Urol. 2003; 10(4): 1950–1951.
  9. Jain M, Kumari N, Chhabra P, et al. Localized amyloidosis of urinary bladder: a diagnostic dilemma. Indian J Pathol Microbiol. 2008; 51(2): 247–249.

Regulations

Important: This website uses cookies. More >>

The cookies allow us to identify your computer and find out details about your last visit. They remembering whether you've visited the site before, so that you remain logged in - or to help us work out how many new website visitors we get each month. Most internet browsers accept cookies automatically, but you can change the settings of your browser to erase cookies or prevent automatic acceptance if you prefer.