Τίτλος:
Biopsy-proven resolution of renal light-chain deposition disease after autologous stem cell transplantation
Γλώσσες Τεκμηρίου:
Αγγλικά
Περίληψη:
Light-chain deposition disease (LCDD) is caused by an underlying clonal plasma cell dyscrasia in which monoclonal immunoglobulin light chains (LCs) are deposited in tissues, resulting in varying degrees of organ dysfunction. Autologous stem cell transplantation (ASCT) has been reported to stabilize renal function in patients with LCDD, but currently, no evidence of histopathologic resolution of LC deposition after ASCT exists. We present a patient, with severe renal dysfunction due to LCDD, who was treated with high-dose melphalan and ASCT that resulted in a significant and extended period of improved renal function. Four years after the initial improvement, the patient developed nephrotic range proteinuria, without any evidence of relapse of the plasma cell dyscrasia. At that time, a repeat renal biopsy showed complete resolution of LC depositions and development of extensive glomerulosclerosis, thus explaining proteinuria. To the best of our knowledge, this is the first report of a biopsy-proven resolution of renal LCDD following ASCT. A timely application of ASCT should be considered in LCDD to prevent deterioration of renal function in the long run. © 2010 The Author.
Συγγραφείς:
Petrakis, I.
Stylianou, K.
Mavroeidi, V.
Vardaki, E.
Stratigis, S.
Stratakis, S.
Xylouri, I.
Perakis, C.
Petraki, C.
Nakopoulou, L.
Daphnis, E.
Περιοδικό:
Nephrology Dialysis Transplantation
Λέξεις-κλειδιά:
creatinine; enalapril; immunoglobulin light chain; melphalan; valsartan, adult; article; autologous stem cell transplantation; case report; clinical feature; creatinine blood level; drug megadose; fatigue; female; glomerulosclerosis; histopathology; human; hypertension; kidney biopsy; kidney disease; leg edema; light chain deposition disease; plasma cell dyscrasia; priority journal; proteinuria; symptom; tachycardia; treatment outcome, Biopsy; Female; Humans; Immunoglobulin Light Chains; Kidney; Kidney Diseases; Middle Aged; Paraproteinemias; Stem Cell Transplantation; Transplantation, Autologous
