title: Primary amyloidosis of the kidney.
abstract: Primary (AL) amyloidosis is a lethal form of plasma cell dyscrasia characterized by monoclonal immunoglobulin light chain deposition causing organ dysfunction. The median age for AL amyloidosis incidence is 64 years, with less than 1% occurring under age 40. The kidney is frequently involved in AL amyloidosis; nephrotic syndrome and/or end stage renal disease (ESRD) occur at presentation in about two-thirds of the patients. High-dose melphalan with autologous peripheral blood stem cell transplantation (ASCT) is an effective treatment for systemic primary amyloidosis. This procedure is, however, associated with substantial toxicity and mortality, particularly if the heart is involved. Here we report a case of primary AL amyloidosis in a 40 year old male who presented with a single-organ involvement (e.g.: kidney) that progressed rapidly to ESRD. Melphalan and ASCT resulted in a complete hematological recovery. This case highlights the possibility that single organ involvement and young age may be associated with better prognosis and improved survival.
authors.affiliation: Division of Nephrology, Department of Internal Medicine, The University of Kansas Medical Center, Kansas City, KS, USA.
authors.forename: Shahzad
authors.initials: S
authors.lastname: Shafique
authors.forename: James
authors.initials: J
authors.lastname: Wetmore
authors.forename: Ammar
authors.initials: A
authors.lastname: Almehmi
mesh_terms: D000328:Adult; D000686:Amyloidosis / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy*; D006801:Humans; D007676:Kidney Failure, Chronic / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy*; D008297:Male; D008558:Melphalan / Q000008:administration & dosage; D019653:Myeloablative Agonists / Q000008:administration & dosage; D033581:Stem Cell Transplantation / Q000379:methods
chemical_list: D019653:Myeloablative Agonists; D008558:Melphalan