Analysis of Paraproyein Deposition in the Kidney: Therapeutic Approach to Renal Amyloidosis
Systemic amyloidosis is almost invariably fatal, with death usually attributable to renal failure or cardiac disease. Recently, it has become possible to identify the major classes of amyloid fibril and the origin of the fibril protein precurcer. However, the mechanism of renal accumulation and removal of paraprotein (PP) has not been fully understood. Proximal tubuli have an important role in absorption and metabolism of amyloid fibril. For example β2-microgrobulin increases in the serum of renal failure and causes amyloidosis especially in heamodialysis patients. Additionally, Fanconi’s syndrome frequently occurs in patiens with Bence Jone’s (kappa type) amyloidosis. Eleven cases of renal amyloidosis were experienced in our center. Two cases of them were difficult to determine the origin of fibril as described previously (XIth European Congress of Pathology, Prague, 1987). However, another 6 cases were AL type and 3 cases were AA type. The purpose of this study was: 1) measurement of serum level of AL and AA proteins by immunological method, 2) clinical approach to removing AL and AA protein from serum and/or tissue in patients with amyloidosis. The results of this study may contribute to elucidating the clinical treatment and opening the key of mechanisms of renal amyloidosis.
KeywordsFree Light Chain Renal Amyloidosis Proximal Tubuli Serum Free Light Chain Plasma Exchange Therapy
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