β₂-Microglobulin-Derived Amyloid Deposition in Acquired Cystic Disease of the Kidney with Renal Cell Carcinoma

Metastasizing Renal Cell Carcinoma and Acquired Renal Cystic Disease in a Hemodialysis Patient

“…The increase in the incidence of the disease with the evolu tionary period of renal insufficiency and the period spent undergoing dialysis [7,8,17,18], and likewise the regres-sion of the disease following renal transplantation [ 19] would support the hypothesis of the existence of a nondialysable renotropic factor [21 ] which would condition the hyperplasia of the tubular epithelium and thus facil itate the formation of cysts [22], The theory that the cysts are due to tubular obstruction by oxylate crystals is also plausible [23]. Finally, the presence of ß2-microglobulin on the walls of the cysts, and its participation in the composition of stones in hemodialysis patients, estab lishes links with the amyloid degeneration of these pa tients, and tubular obstruction mechanisms can be justi fied [12].…”

“…The incidence of adenomas amongst the total number of patients undergoing dialysis is 7.5% [2,3,[8][9][10][11][12]16] and 21 % among patients with ARCD. These percentages are 2.7 and 7.7%, respectively, for those patients with renal cell carcinomas.…”

Uremic Acquired Renal Cystic Disease

“…Almost all acquired cysts originate from the proximal tubules [40], as demonstrated by scanning electron microscopy [30], lectin conjugate reactivity [39,40], cyst fluid analysis [46] and detection of hippuran excretion into the cysts [41], p2-Microglobulin deposition is known to occur around the cyst wall [47] and in vessel walls. In addition, protein stones form in the collecting ducts, and oxalate crystals are observed in the cyst walls and interstitium [1], How ever, it is not known whether these Pi-microglobulin and oxalate accumulations are related to the development of acquired cysts or not [47,48].…”

Amyloidosis

“…31]. Periarticular bone cysts and cystic disease of the kidney have been reported [28,43,86,103], There exists some controversy about the dialysisrelated amyloid regarding birefringence under polarized light, ultrastructure and existence of the P component [116,162], However, in some studies, the P2-M showed a fibrillar ultrastructure typical of amyloid, bound Congo red, and gave green birefringence [39],…”