Amiloidosi polidistrettuale. Implicazioni urologiche

Abstract

Amyloidosis covers a group of morbid processes which have the common characteristic of amyloid being deposited in an extracellular site of one or more organs. The mechanisms causing amyloidogenesis are still unknown. Involvement of the urinary apparatus is more frequent with primary amyloidosis and myeloma (AL form) as well as inflammatory or neoplastic diseases (AA form). The disease in the kidney is characterised by proteinuria progressing to the nephrotic syndrome and renal failure, while frank hematuria is typical of the disease in the bladder. In our case emergency cystectomy was performed because of severe anemia consequent to hematuria. Prodromal symptoms were asthenia, loss of weight, purpura and subsequent to involvement of the urinary tract, also gastroenteric, cardiac, neurovegetative, articular and thyroid disorders typical of multi-district involvement. Diagnosis is confirmed only after the histological examination and average survival rate is 20 months. There is no fully effective therapy.