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Early diagnosis, chemotherapy response influence renal survival in LCDD
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Early diagnosis and treatment of light chain deposition disease is essential to delay progression to end-stage renal disease and prevent recurrence following kidney transplantation, according to prospective study results.
Light chain deposition disease (LCDD) occurs when monotypic immunoglobulin light chains are deposited in the kidney, resulting in renal dysfunction. Patients who underwent renal transplantation have generally experienced poor outcomes, according to study background.
Julian D. Gillmore, MBBS, MD, PhD, senior lecturer at University College London’s National Amyloidosis Center, and colleagues prospectively followed a cohort of patients with biopsy-confirmed LCDD to observe clinical outcomes.
The study included data from 53 patients (median age, 56 years; range, 29-78). Investigational methods included a 6-minute walking test, electrocardiography, detailed echocardiography, serological cardiac, liver and bone function marker testing, and urine biochemistry.
Median follow-up was 6.2 years (range, 1.1-14).
The researchers observed a median renal survival from diagnosis of 5.4 years, with an estimated patient survival of 14 years. Sixty-four percent of patients remained alive at time of reporting.
Renal outcome appeared strongly associated with hematologic response to chemotherapy, with a mean glomerular filtration rate (GFR) improvement of 6.1 mL/min per year among patients achieving a complete response or very good partial response (n = 21). A majority of responding patients remained dialysis independent, whereas patients who achieved partial response or no response experienced a mean GFR loss of 6.5 mL/min per year (P < .009).
A significant proportion of patients who did not respond to chemotherapy developed end-stage renal disease (P = .005).
Seven patients underwent renal transplant; among patients whose underlying clonal disorder remained in sustained remission, there was no recurrence of LCDD for up to 9.7 years.
Nineteen patients died during follow-up. Causes of death included infection (n = 6), ischemic heart disease (n = 4), end-stage renal failure (n = 3), congestive cardiac failure (n = 2), multiple myeloma (n = 2), gastrointestinal hemorrhage (n = 1) and cerebrovascular accident (n = 1).
“Patient survival as well as tolerance of high-dose chemotherapy appears to be substantially better in LCDD than in systemic AL amyloidosis,” Gillmore and colleagues wrote. “LCDD should be aggressively treated with chemotherapy, since achieving a hematologic complete response or very good partial response prolongs renal survival, even if advanced renal impairment has supervened, while also preventing renal allograft failure from recurrent LCDD among those who receive renal transplant.” – by Cameron Kelsall
Disclosure: The researchers report no relevant financial disclosures.
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