Biopsy-proven resolution of renal light-chain deposition disease after autologous stem cell transplantation.
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.
Petrakis
Ioannis
I
Department of Nephrology, Heraklion University Hospital, Crete, Greece.
Stylianou
Kostas
K
Mavroeidi
Vasiliki
V
Vardaki
Eleftheria
E
Stratigis
Spyridon
S
Stratakis
Stavros
S
Xylouri
Irene
I
Perakis
Constantinos
C
Petraki
Constantina
C
Nakopoulou
Lydia
L
Daphnis
Eugene
E
D001706:Biopsy; D005260:Female; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000378:metabolism*; D007668:Kidney / Q000276:immunology / Q000473:pathology / Q000503:physiopathology; D007674:Kidney Diseases / Q000276:immunology* / Q000473:pathology / Q000503:physiopathology / Q000628:therapy*; D008875:Middle Aged; D010265:Paraproteinemias / Q000276:immunology* / Q000473:pathology / Q000503:physiopathology / Q000628:therapy*; D033581:Stem Cell Transplantation; D014182:Transplantation, Autologous
D007147:Immunoglobulin Light Chains