![]() Median follow-up for the whole cohort was 6.2 years (range, 1.1-14.0 years). No monoclonal protein detected by immunofixation (n) Monoclonal protein in serum detected by immunofixation (n) Median (range) pathogenic free light chain concentration (mg/L) Mean (range) urinary protein loss (g/24 hours)Ībnormal serum FLC concentration and ratio (n) 1, 7 Hepatic, cardiac, and neural deposits have also been documented however, and need to be considered in all newly diagnosed patients with renal LCDD. 4 Because LCs are filtered by the glomeruli, reabsorbed in proximal tubules by receptor-mediated endocytosis, and degraded in tubular cells by lysosomal enzymes, 4-6 the kidney is the principal target for LC deposition, and renal involvement and dysfunction usually dominate the clinical disease course. 2, 3 Clinical manifestations of LCDD vary, depending on which organs are involved. ![]() 1 The most commonly diagnosed monoclonal immunoglobulin deposition disease is light chain deposition disease (LCDD) in which monoclonal immunoglobulin light chains (LCs) are deposited, the others being heavy chain deposition disease and light and heavy chain deposition disease. Monoclonal immunoglobulin deposition disease is a group of multisystem disorders characterized by deposition of monoclonal immunoglobulin light or heavy chains in various organs. To participate in this journal CME activity: (1) review the learning objectives and author disclosures (2) study the education content (3) take the post-test with a 75% minimum passing score and complete the evaluation at and (4) view/print certificate. Physicians should claim only the credit commensurate with the extent of their participation in the activity.Īll other clinicians completing this activity will be issued a certificate of participation. Medscape, LLC designates this Journal-based CME activity for a maximum of 1.0 AMA PRA Category 1 Credit(s)™. Medscape, LLC is accredited by the ACCME to provide continuing medical education for physicians. This activity has been planned and implemented in accordance with the Essential Areas and policies of the Accreditation Council for Continuing Medical Education through the joint providership of Medscape, LLC and the American Society of Hematology. Medscape Continuing Medical Education online This study highlights the need to diagnose and treat LCDD early and to target at least a hematologic VGPR with chemotherapy, even among patients with advanced renal dysfunction, to delay progression to ESRD and prevent recurrence of LCDD in the renal allografts of those who subsequently receive a kidney transplant. Seven patients received a renal transplant, and among those whose underlying clonal disorder was in sustained remission, there was no recurrence of LCDD up to 9.7 years later. 009), most of whom developed end-stage renal disease (ESRD P =. There was a strong association between hematologic response to chemotherapy and renal outcome, with a mean improvement in glomerular filtration rate (GFR) of 6.1 mL/min/year among those achieving a complete or very good partial hematologic response (VGPR) with chemotherapy, most of whom remained dialysis independent, compared with a mean GFR loss of 6.5 mL/min/year among those achieving only a partial or no hematologic response ( P <. ![]() Sixty-two percent of patients required dialysis, and median survival from commencement of dialysis was 5.2 years. ![]() Median renal survival from diagnosis by Kaplan-Meier analysis was 5.4 years, and median estimated patient survival was 14.0 years 64% of patients were alive at censor. Median age at diagnosis was 56 years, and patients were followed for a median of 6.2 years (range, 1.1-14.0 years). ![]() Fifty-three patients with biopsy-proven LCDD were prospectively followed at the UK National Amyloidosis Center. Light chain deposition disease (LCDD) is characterized by the deposition of monotypic immunoglobulin light chains in the kidney, resulting in renal dysfunction. ![]()
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