Senile Systemic Amyloidosis Presenting as Hematuria: A Rare Presentation and Review of Literature
Joint Authors
Jayakrishnan, Thejus T.
Shah, Deep
Mewawalla, Prerna
Kamran, Amir
Guha, Aritra
Salman Faisal, Muhammad
Source
Issue
Vol. 2020, Issue 2020 (31 Dec. 2020), pp.1-5, 5 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2020-01-03
Country of Publication
Egypt
No. of Pages
5
Main Subjects
Abstract EN
Introduction.
Senile systemic amyloidosis is a multisystem disease where wild-type insoluble transthyretin (ATTRwt) protein gets deposited in the tissues leading to organ dysfunction.
Methodology.
We present the case of a patient who presented with hematuria and bladder involvement by ATTRwt amyloidosis who ultimately died of multiorgan failure.
Results.
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.
Conclusion.
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.
American Psychological Association (APA)
Jayakrishnan, Thejus T.& Kamran, Amir& Shah, Deep& Guha, Aritra& Salman Faisal, Muhammad& Mewawalla, Prerna. 2020. Senile Systemic Amyloidosis Presenting as Hematuria: A Rare Presentation and Review of Literature. Case Reports in Medicine،Vol. 2020, no. 2020, pp.1-5.
https://search.emarefa.net/detail/BIM-1148533
Modern Language Association (MLA)
Jayakrishnan, Thejus T.…[et al.]. Senile Systemic Amyloidosis Presenting as Hematuria: A Rare Presentation and Review of Literature. Case Reports in Medicine No. 2020 (2020), pp.1-5.
https://search.emarefa.net/detail/BIM-1148533
American Medical Association (AMA)
Jayakrishnan, Thejus T.& Kamran, Amir& Shah, Deep& Guha, Aritra& Salman Faisal, Muhammad& Mewawalla, Prerna. Senile Systemic Amyloidosis Presenting as Hematuria: A Rare Presentation and Review of Literature. Case Reports in Medicine. 2020. Vol. 2020, no. 2020, pp.1-5.
https://search.emarefa.net/detail/BIM-1148533
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1148533