Abstract
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.
References
Feb 1, 1976·The American Journal of Medicine·R E RandallW J Still
Oct 1, 1995·Annals of Internal Medicine·X MarietteJ C Brouet
Oct 1, 1995·Annals of Internal Medicine·D A Blandino
Oct 1, 1994·Kidney International·J L Preud'hommeM Cogné
Feb 29, 2000·American Journal of Kidney Diseases : the Official Journal of the National Kidney Foundation·A KomatsudaA B Miura
May 15, 2001·Laboratory Investigation; a Journal of Technical Methods and Pathology·W J RussellG A Herrera
Jun 26, 2001·Journal of the American Society of Nephrology : JASN·Julie LinVivette D D'Agati
Jul 18, 2002·Nephron·Osamu Hotta, Yoshio Taguma
Dec 5, 2003·American Journal of Kidney Diseases : the Official Journal of the National Kidney Foundation·Claudio PozziFrancesco Locatelli
Jan 14, 2004·Kidney International·Bruno RoyerJean-Paul Fermand
Mar 3, 2004·Laboratory Investigation; a Journal of Technical Methods and Pathology·Jiamin TengGuillermo A Herrera
Aug 3, 2004·Laboratory Investigation; a Journal of Technical Methods and Pathology·John KeelingGuillermo A Herrera
Aug 28, 2004·American Journal of Kidney Diseases : the Official Journal of the National Kidney Foundation·Frank FirkinHilton Gock
Sep 17, 2005·Kidney International·John Keeling, Guillermo A Herrera
Jul 25, 2006·Bone Marrow Transplantation·K WeichmanV Sanchorawala
Aug 21, 2007·Clinical Journal of the American Society of Nephrology : CJASN·Pierre RoncoPierre Aucouturier
Aug 19, 2007·Clinical Journal of the American Society of Nephrology : CJASN·Nelson LeungMorie A Gertz
Jan 8, 2008·Nephrology, Dialysis, Transplantation : Official Publication of the European Dialysis and Transplant Association - European Renal Association·Elizabeth C LorenzNelson Leung
Mar 7, 2008·Kidney International·M W Taal, B M Brenner
Dec 11, 2008·Haematologica·Efstathios KastritisMeletios A Dimopoulos
Citations
Mar 29, 2012·Renal Failure·Stephen I Rifkin, Samuel S Weinstein
Oct 18, 2016·Internal Medicine·Toshiharu UenoYoshifumi Ubara
Aug 19, 2015·Terapevticheskiĭ arkhiv·I G RekhtinaE N Denisova
Jan 25, 2021·BMC Nephrology·Takafumi TsushimaKosei Matsue
Jun 24, 2021·Curēus·Yoshinosuke ShimamuraYasuo Sakurai