Gross hematuria: An unusual presenting symptom of systemic wild-type transthyretin amyloidosis

Abstract

Amyloidosis of the urinary bladder is a rare cause of gross hematuria. In patients with systemic amyloidosis, identification is nearly always related to cardiac complaints; urologic presenting symptoms are extremely uncommon. We present a 77-year-old male patient with painless gross hematuria ultimately found to be secondary to systemic wild-type transthyretin amyloidosis. He underwent transurethral resection of the bladder lesion and was initiated on transthyretin stabilizing medication. In the 6 months since starting treatment, the patient has had no further episodes of gross hematuria, but will require cystoscopic surveillance for evidence of recurrence or concomittment urothelial carcinoma.

A Case of Isolated Renal Light Chain Amyloidosis With Hematuria and Low Complement Levels

“…Although, in our case, the patient had a prior history of chronic kidney disease and drug use, these risk factors would predispose the patient to nephrotic range proteinuria and not specifically AL amyloidosis [ 11 ]. However, a few cases of transthyretin amyloid (ATTR)-associated amyloidosis have been documented in older individuals presenting with systemic manifestations [ 8 , 10 ]. Isolated renal AL amyloidosis was mostly diagnosed in females [ 6 ].…”

“…The presenting symptom in our study was abdominal pain; however, patients in previous reports also presented with generalized weakness [ 6 ], night sweats [ 6 ], rashes [ 6 ], hematuria [ 5 , 7 , 8 , 9 ], and multiorgan failure [ 8 ]. Hematuria was most commonly found in cases with senile wild-type systemic amyloidosis [ 8 ], transthyretin amyloidosis of the urinary bladder [ 10 ], AA amyloidosis [ 6 ], and amyloidosis with concomitant crescentic glomerulonephritis [ 7 ]. In previous studies, proteinuria and albumin creatinine ratios usually ranged around 1-2 g/day [ 6 , 9 ].…”

“…Hematuria as a presenting feature of isolated renal AL amyloidosis is relatively uncommon. To date, there are seven reports discussing patients with hematuria who were later diagnosed with either isolated or systemic amyloidosis [5][6][7][8][9][10]. Of these seven reports, three articles discussed hematuria as a presenting feature of AL amyloidosis [5,7,8].…”