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000170085 1001_ $$00000-0003-3829-0499$$aBasset, Marco$$b0
000170085 245__ $$aLenalidomide and dexamethasone in relapsed/refractory immunoglobulin light chain (AL) amyloidosis: results from a large cohort of patients with long follow-up.
000170085 260__ $$aOxford [u.a.]$$bWiley-Blackwell$$c2021
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000170085 500__ $$a2021 Oct;195(2):230-243
000170085 520__ $$aLenalidomide and dexamethasone (RD) is a standard treatment in relapsed/refractory immunoglobulin light chain (AL) amyloidosis (RRAL). We retrospectively investigated toxicity, efficacy and prognostic markers in 260 patients with RRAL. Patients received a median of two prior treatment lines (68% had been bortezomib-refractory; 33% had received high-dose melphalan). The median treatment duration was four cycles. The 3-month haematological response rate was 31% [very good haematological response (VGHR) in 18%]. The median follow-up was 56·5 months and the median overall survival (OS) and haematological event-free survival (haemEFS) were 32 and 9 months. The 2-year dialysis rate was 15%. VGHR resulted in better OS (62 vs. 26 months, P < 0·001). Cardiac progression predicted worse survival (22 vs. 40 months, P = 0·027), although N-terminal prohormone of brain natriuretic peptide (NT-proBNP) increase was frequently observed. Multivariable analysis identified these prognostic factors: NT-proBNP for OS [hazard ratio (HR) 1·71; P < 0·001]; gain 1q21 for haemEFS (HR 1·68, P = 0·014), with a trend for OS (HR 1·47, P = 0·084); difference between involved and uninvolved free light chains (dFLC) and light chain isotype for OS (HR 2·22, P < 0·001; HR 1·62, P = 0·016) and haemEFS (HR 1·88, P < 0·001; HR 1·59, P = 0·008). Estimated glomerular filtration rate (HR 0·71, P = 0·004) and 24-h proteinuria (HR 1·10, P = 0·004) were prognostic for renal survival. In conclusion, clonal and organ biomarkers at baseline identify patients with favourable outcome, while VGHR and cardiac progression define prognosis during RD treatment.
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000170085 650_7 $$2Other$$aAL amyloidosis
000170085 650_7 $$2Other$$abiomarkers
000170085 650_7 $$2Other$$again 1q21
000170085 650_7 $$2Other$$alenalidomide
000170085 650_7 $$2Other$$aprognosis
000170085 7001_ $$aKimmich, Christoph R$$b1
000170085 7001_ $$0P:(DE-He78)0d054b6843ace36d1c965b6cb938d1c9$$aSchreck, Nicholas$$b2$$udkfz
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000170085 7001_ $$aDittrich, Tobias$$b4
000170085 7001_ $$aVeelken, Kaya$$b5
000170085 7001_ $$aGoldschmidt, Hartmut$$b6
000170085 7001_ $$aSeckinger, Anja$$b7
000170085 7001_ $$aHose, Dirk$$b8
000170085 7001_ $$aJauch, Anna$$b9
000170085 7001_ $$aMüller-Tidow, Carsten$$b10
000170085 7001_ $$0P:(DE-He78)e15dfa1260625c69d6690a197392a994$$aBenner, Axel$$b11$$udkfz
000170085 7001_ $$aHegenbart, Ute$$b12
000170085 7001_ $$aSchönland, Stefan O$$b13
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