Basset, Marco;
Kimmich, Christoph R.;
Schreck, Nicholas;
Krzykalla, Julia;
Dittrich, Tobias;
Veelken, Kaya;
Goldschmidt, Hartmut;
Seckinger, Anja;
Hose, Dirk;
Jauch, Anna;
Müller‐Tidow, Carsten;
Benner, Axel;
Hegenbart, Ute;
Schönland, Stefan O.
Lenalidomide and dexamethasone in relapsed/refractory immunoglobulin light chain (AL) amyloidosis: results from a large cohort of patients with long follow‐up
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Medientyp:
E-Artikel
Titel:
Lenalidomide and dexamethasone in relapsed/refractory immunoglobulin light chain (AL) amyloidosis: results from a large cohort of patients with long follow‐up
Beschreibung:
<jats:title>Summary</jats:title><jats:p>Lenalidomide and dexamethasone (RD) is a standard treatment in relapsed/refractory immunoglobulin light chain (AL) amyloidosis (RRAL). We retrospectively investigated toxicity, efficacy and prognostic markers in 260 patients with RRAL. Patients received a median of two prior treatment lines (68% had been bortezomib‐refractory; 33% had received high‐dose melphalan). The median treatment duration was four cycles. The 3‐month haematological response rate was 31% [very good haematological response (VGHR) in 18%]. The median follow‐up was 56·5 months and the median overall survival (OS) and haematological event‐free survival (haemEFS) were 32 and 9 months. The 2‐year dialysis rate was 15%. VGHR resulted in better OS (62 vs. 26 months, <jats:italic>P</jats:italic> < 0·001). Cardiac progression predicted worse survival (22 vs. 40 months, <jats:italic>P</jats:italic> = 0·027), although N‐terminal prohormone of brain natriuretic peptide (NT‐proBNP) increase was frequently observed. Multivariable analysis identified these prognostic factors: NT‐proBNP for OS [hazard ratio (HR) 1·71; <jats:italic>P</jats:italic> < 0·001]; gain 1q21 for haemEFS (HR 1·68, <jats:italic>P</jats:italic> = 0·014), with a trend for OS (HR 1·47, <jats:italic>P</jats:italic> = 0·084); difference between involved and uninvolved free light chains (dFLC) and light chain isotype for OS (HR 2·22, <jats:italic>P</jats:italic> < 0·001; HR 1·62, <jats:italic>P</jats:italic> = 0·016) and haemEFS (HR 1·88, <jats:italic>P</jats:italic> < 0·001; HR 1·59, <jats:italic>P</jats:italic> = 0·008). Estimated glomerular filtration rate (HR 0·71, <jats:italic>P</jats:italic> = 0·004) and 24‐h proteinuria (HR 1·10, <jats:italic>P</jats:italic> = 0·004) were prognostic for renal survival. In conclusion, clonal and organ biomarkers at baseline identify patients with favourable outcome, while VGHR and cardiac progression define prognosis during RD treatment.</jats:p>