Senile Systemic Amyloidosis Presenting as Hematuria: A Rare Presentation and Review of Literature.
Journal
Case reports in medicine
ISSN: 1687-9627
Titre abrégé: Case Rep Med
Pays: United States
ID NLM: 101512910
Informations de publication
Date de publication:
2020
2020
Historique:
received:
27
06
2019
revised:
03
12
2019
accepted:
16
12
2019
entrez:
28
1
2020
pubmed:
28
1
2020
medline:
28
1
2020
Statut:
epublish
Résumé
Senile systemic amyloidosis is a multisystem disease where wild-type insoluble transthyretin (ATTRwt) protein gets deposited in the tissues leading to organ dysfunction. We present the case of a patient who presented with hematuria and bladder involvement by ATTRwt amyloidosis who ultimately died of multiorgan failure. The patient was an 82-year-old white male with a history of ischemic cardiomyopathy (ejection fraction (EF): 20-25%), chronic atrial fibrillation, chronic kidney disease (CKD), and carpal tunnel syndrome who presented with acute hematuria, urinary retention, and progressive fatigue. He underwent cystoscopy and bladder biopsy which was positive on congo-red stain diagnostic for amyloidosis. Echocardiogram demonstrated worsening of EF to 10-15% and concentric left ventricle hypertrophy. MRI was not performed due to underlying CKD. His condition deteriorated during the hospital stay, and he developed cardiogenic shock and progressive liver dysfunction. Infectious workup was negative. Meanwhile, the biochemical investigations (serum protein electrophoresis, immunofixation, and urine kappa/lambda chains) ruled out plasma cell dyscrasias. Mass spectrometry analysis of the bladder biopsy specimen confirmed wild-type transthyretin (ATTRwt) amyloidosis consistent with senile systemic amyloidosis. Due to patients' extremely poor prognosis, his family wished to focus on patient's comfort-oriented measures only, and patient passed away shortly thereafter. Senile systemic amyloidosis can rarely present in an atypical fashion such as hematuria. The treatment options are limited in this disease process. Novel therapies are in the early phases of development. Concern also exists that in patients with multiple comorbidities, this entity is under recognized until the more advanced stages.
Identifiants
pubmed: 31983923
doi: 10.1155/2020/5892707
pmc: PMC6964363
doi:
Types de publication
Case Reports
Langues
eng
Pagination
5892707Informations de copyright
Copyright © 2020 Thejus Jayakrishnan et al.
Déclaration de conflit d'intérêts
The authors declare that there are no conflicts of interest.
Références
Jpn J Radiol. 2013 Oct;31(10):693-700
pubmed: 23996116
Eur Heart J. 2015 Oct 7;36(38):2585-94
pubmed: 26224076
Nat Rev Neurol. 2019 Jul;15(7):387-404
pubmed: 31209302
JAMA. 2013 Dec 25;310(24):2658-67
pubmed: 24368466
Blood. 2009 Dec 3;114(24):4957-9
pubmed: 19797517
Amyloid. 2019 Sep;26(3):103-111
pubmed: 31339362
Curr Med Res Opin. 2013 Jan;29(1):77-84
pubmed: 23193943
Eur Heart J. 2019 Dec 1;40(45):3699-3706
pubmed: 31111153
N Engl J Med. 2018 Sep 13;379(11):1007-1016
pubmed: 30145929
EuPA Open Proteom. 2016 Feb 23;11:4-10
pubmed: 29900105
Circulation. 2016 Jun 14;133(24):2404-12
pubmed: 27143678
Amyloid. 2017 Dec;24(4):253-255
pubmed: 29052438
Amyloid. 2016;23(1):58-63
pubmed: 26852880
Amyloid. 2015;22(2):79-83
pubmed: 26017328
Rev Esp Cardiol (Engl Ed). 2016 Oct;69(10):923-930
pubmed: 27291669
Rev Esp Cardiol (Engl Ed). 2016 Oct;69(10):888-889
pubmed: 27514622
Eur Heart J. 2015 Oct 7;36(38):2595-7
pubmed: 26224073
Blood. 2012 Jan 12;119(2):488-93
pubmed: 22106346
Int J Health Sci (Qassim). 2011 Jul;5(2):181-5
pubmed: 23267295
Clin Med Insights Cardiol. 2015 Jan 05;8(Suppl 1):39-44
pubmed: 25628512
J Community Hosp Intern Med Perspect. 2014 Nov 25;4(5):25500
pubmed: 25432650
Circ Heart Fail. 2016 Sep;9(9):
pubmed: 27618855
Intern Med. 2016;55(9):1109-15
pubmed: 27150863
Arch Cardiovasc Dis. 2018 Oct;111(10):582-590
pubmed: 29709420
Ther Adv Cardiovasc Dis. 2013 Aug;7(4):224-7
pubmed: 23868674
Amyloid. 2018 Dec;25(4):215-219
pubmed: 30614283
Cardiology. 2016;135(4):216-220
pubmed: 27522614
Rom J Morphol Embryol. 2017;58(1):201-206
pubmed: 28523319
JACC Heart Fail. 2017 Aug;5(8):617
pubmed: 28774400