Life-threatening hematuria in a hemodialysis patient with systemic light-chain amyloidosis

Clin Chim Acta. 2015 Dec 7;451(Pt B):180-2. doi: 10.1016/j.cca.2015.09.027. Epub 2015 Oct 21.

Abstract

Background: Direct amyloid invasion of prostate tissue resulting in massive bleeding may be fatal, and rapid diagnosis is difficult.

Case report: 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.

Keywords: Amyloid; Hematuria; Light-chain amyloidosis; Prostate bleeding.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Amyloidosis / blood*
  • Amyloidosis / complications
  • Amyloidosis / pathology
  • Hematuria / blood*
  • Hematuria / etiology
  • Hematuria / pathology
  • Hemorrhage / blood
  • Hemorrhage / etiology
  • Hemorrhage / pathology
  • Humans
  • Immunoglobulin Light Chains / blood*
  • Male
  • Microscopy, Electron
  • Renal Dialysis

Substances

  • Immunoglobulin Light Chains