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

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

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.

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.