title: Progressive renal light chain amyloidosis with the absence of detectable free monoclonal light chains after an autologous hematopoietic stem cell transplant for amyloid light chain amyloidosis.
abstract: Amyloid light chain amyloidosis involving the kidneys is not uncommon in patients with monoclonal gammopathy. The mainstay of treatment of amyloid light chain amyloidosis is autologous hematopoietic stem cell transplantation. Evidence that the autologous hematopoietic stem cell transplantation has been successful is the absence of free monoclonal light chains in serum and urine. Herein, we report 2 cases of progressive renal amyloid light chain amyloidosis after autologous hematopoietic stem cell transplantation, documented by kidney biopsy, despite the absence of monoclonal protein in the serum and urine. Kidney function declined progressively in both patients. During that time, numerous immunofixation and protein electrophoresis test results were negative for monoclonal protein, both in serum and urine, concealing the progression of the amyloidosis. We conclude that close monitoring of kidney function is warranted following autologous hematopoietic stem cell transplantation in patients with amyloid light chain amyloidosis, even with negative results from monoclonal protein testing. Unexplained, worsening renal function warrants a kidney biopsy to assess whether retreatment of the monoclonal gammopathy is indicated.
authors.affiliation: Department of Pathology, Ohio State University, Columbus, USA.
authors.forename: Rachel M
authors.initials: RM
authors.lastname: Roth
authors.forename: Don
authors.initials: D
authors.lastname: Benson
authors.forename: Lee A
authors.initials: LA
authors.lastname: Hebert
authors.forename: Michael G
authors.initials: MG
authors.lastname: Bissell
authors.forename: Anjali A
authors.initials: AA
authors.lastname: Satoskar
authors.forename: Tibor
authors.initials: T
authors.lastname: Nadasdy
authors.forename: Sergey V
authors.initials: SV
authors.lastname: Brodsky
mesh_terms: D000369:Aged, 80 and over; D000686:Amyloidosis / Q000276:immunology / Q000473:pathology* / Q000628:therapy; D001706:Biopsy; D003404:Creatinine / Q000097:blood; D017809:Fatal Outcome; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000276:immunology; D007668:Kidney / Q000378:metabolism / Q000473:pathology*; D007674:Kidney Diseases / Q000276:immunology / Q000473:pathology* / Q000628:therapy; D007677:Kidney Function Tests; D008297:Male; D008875:Middle Aged; D010265:Paraproteinemias / Q000276:immunology / Q000473:pathology* / Q000628:therapy; D058225:Plaque, Amyloid / Q000378:metabolism; D011507:Proteinuria; D014182:Transplantation, Autologous
chemical_list: D007147:Immunoglobulin Light Chains; D003404:Creatinine