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@ARTICLE{Basset:170085,
author = {M. Basset and C. R. Kimmich and N. Schreck$^*$ and J.
Krzykalla$^*$ and T. Dittrich and K. Veelken and H.
Goldschmidt and A. Seckinger and D. Hose and A. Jauch and C.
Müller-Tidow and A. Benner$^*$ and U. Hegenbart and S. O.
Schönland},
title = {{L}enalidomide and dexamethasone in relapsed/refractory
immunoglobulin light chain ({AL}) amyloidosis: results from
a large cohort of patients with long follow-up.},
journal = {British journal of haematology},
volume = {195},
number = {2},
issn = {1365-2141},
address = {Oxford [u.a.]},
publisher = {Wiley-Blackwell},
reportid = {DKFZ-2021-01748},
pages = {230-243},
year = {2021},
note = {2021 Oct;195(2):230-243},
abstract = {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.},
keywords = {AL amyloidosis (Other) / biomarkers (Other) / gain 1q21
(Other) / lenalidomide (Other) / prognosis (Other)},
cin = {C060},
ddc = {610},
cid = {I:(DE-He78)C060-20160331},
pnm = {313 - Krebsrisikofaktoren und Prävention (POF4-313)},
pid = {G:(DE-HGF)POF4-313},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:34341985},
doi = {10.1111/bjh.17685},
url = {https://inrepo02.dkfz.de/record/170085},
}