title: Life-threatening hematuria in a hemodialysis patient with systemic light-chain amyloidosis.
abstract: BACKGROUND
  Direct amyloid invasion of prostate tissue resulting in massive bleeding may be fatal, and rapid diagnosis is difficult.
  
  
  METHODS
  A 71-y-old male undergoing regular hemodialysis with primary light-chain (AL) amyloidosis was admitted due to gross hematuria for 2 days. Cystoscopy revealed oozing from the prostatic urethra. Therefore, electrocauterization was performed, and his symptoms resolved. Unfortunately, he experienced recurrent massive hematuria 3 months later. Tests for serum D-dimer and fibrin degradation products were both positive. Followed serum factor X level was low at 5.4%. Gross hematuria persisted despite of blood transfusions, desmopressin, and vitamin K therapy. Emergent cystoscopy revealed oozing from the prostatic urethra, as was found previously. Therefore, electrocauterization and transurethral resection of the prostate were performed. Analysis of a biopsy specimen of prostate demonstrated strong amyloid deposition in the vascular and perivascular regions. Electron microscopy showed relatively straight fibrils with diameters of 7-10nm in the perivascular region. Gross hematuria subsided then, and no recurrence was noted at a 6-month follow-up.
  
  
  CONCLUSIONS
  Systemic AL amyloidosis can cause potentially life-threatening hemorrhage. Hemostatic defects and direct invasion with amyloid angiopathy are main pathogenic factors. Timely surgical intervention may be imperative.
authors.affiliation: Division of Nephrology, Department of Internal Medicine, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan; Department of Internal Medicine, Taoyuan Armed Forces General Hospital, Taiwan.
authors.forename: Po-Jen
authors.initials: PJ
authors.lastname: Hsiao
authors.affiliation: Division of Nephrology, Department of Internal Medicine, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan; Department of Internal Medicine, Taoyuan Armed Forces General Hospital, Taiwan.
authors.forename: Wen-Fang
authors.initials: WF
authors.lastname: Chiang
authors.affiliation: Department of Pathology, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan.
authors.forename: Tai-Kuang
authors.initials: TK
authors.lastname: Chao
authors.affiliation: Division of Nephrology, Department of Internal Medicine, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan. Electronic address: l521116@ndmctsgh.edu.tw.
authors.forename: Shih-Hua
authors.initials: SH
authors.lastname: Lin
mesh_terms: D000368:Aged; D000686:Amyloidosis / Q000097:blood* / Q000150:complications / Q000473:pathology; D006417:Hematuria / Q000097:blood* / Q000209:etiology / Q000473:pathology; D006470:Hemorrhage / Q000097:blood / Q000209:etiology / Q000473:pathology; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000097:blood*; D008297:Male; D008854:Microscopy, Electron; D006435:Renal Dialysis
chemical_list: D007147:Immunoglobulin Light Chains
keywords: Amyloid; Hematuria; Light-chain amyloidosis; Prostate bleeding