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Lenalidomide and dexamethasone in relapsed/refractory immunoglobulin light chain (AL) amyloidosis: results from a large cohort of patients with long follow-up

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...

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Main Authors: Basset, Marco (Author) , Kimmich, Christoph (Author) , Schreck, Nicholas (Author) , Krzykalla, Julia (Author) , Dittrich, Tobias (Author) , Veelken, Kaya (Author) , Goldschmidt, Hartmut (Author) , Seckinger, Anja (Author) , Hose, Dirk (Author) , Jauch, Anna (Author) , Müller-Tidow, Carsten (Author) , Benner, Axel (Author) , Hegenbart, Ute (Author) , Schönland, Stefan (Author)
Format: Article (Journal)
Language:English
Published: 3 August 2021
In: British journal of haematology
Year: 2021, Volume: 195, Issue: 2, Pages: 230-243
ISSN:1365-2141
DOI:10.1111/bjh.17685
Online Access:Verlag, kostenfrei, Volltext: https://doi.org/10.1111/bjh.17685
Verlag, kostenfrei, Volltext: https://onlinelibrary.wiley.com/doi/abs/10.1111/bjh.17685
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Author Notes:Marco Basset, Christoph R. Kimmich, Nicholas Schreck, Julia Krzykalla, Tobias Dittrich, Kaya Veelken, Hartmut Goldschmidt, Anja Seckinger, Dirk Hose, Anna Jauch, Carsten Müller-Tidow, Axel Benner, Ute Hegenbart, and Stefan O. Schönland
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Summary: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, P < 0·001). Cardiac progression predicted worse survival (22 vs. 40 months, P = 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; P < 0·001]; gain 1q21 for haemEFS (HR 1·68, P = 0·014), with a trend for OS (HR 1·47, P = 0·084); difference between involved and uninvolved free light chains (dFLC) and light chain isotype for OS (HR 2·22, P < 0·001; HR 1·62, P = 0·016) and haemEFS (HR 1·88, P < 0·001; HR 1·59, P = 0·008). Estimated glomerular filtration rate (HR 0·71, P = 0·004) and 24-h proteinuria (HR 1·10, P = 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.
Item Description:Gesehen am 14.08.2023
Physical Description:Online Resource
ISSN:1365-2141
DOI:10.1111/bjh.17685

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