{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,16]],"date-time":"2026-06-16T21:23:56Z","timestamp":1781645036486,"version":"3.54.5"},"reference-count":49,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2021,8,3]],"date-time":"2021-08-03T00:00:00Z","timestamp":1627948800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001659","name":"Deutsche Forschungsgemeinschaft","doi-asserted-by":"publisher","award":["HE 8472\/1\u20101"],"award-info":[{"award-number":["HE 8472\/1\u20101"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001659","name":"Deutsche Forschungsgemeinschaft","doi-asserted-by":"publisher","award":["SCHO 1364\/2\u20101"],"award-info":[{"award-number":["SCHO 1364\/2\u20101"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Br J Haematol"],"published-print":{"date-parts":[[2021,10]]},"abstract":"<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\u2010refractory; 33% had received high\u2010dose melphalan). The median treatment duration was four cycles. The 3\u2010month haematological response rate was 31% [very good haematological response (VGHR) in 18%]. The median follow\u2010up was 56\u00b75\u00a0months and the median overall survival (OS) and haematological event\u2010free survival (haemEFS) were 32 and 9\u00a0months. The 2\u2010year dialysis rate was 15%. VGHR resulted in better OS (62 vs. 26\u00a0months, <jats:italic>P<\/jats:italic>\u00a0&lt;\u00a00\u00b7001). Cardiac progression predicted worse survival (22 vs. 40\u00a0months, <jats:italic>P<\/jats:italic>\u00a0=\u00a00\u00b7027), although N\u2010terminal prohormone of brain natriuretic peptide (NT\u2010proBNP) increase was frequently observed. Multivariable analysis identified these prognostic factors: NT\u2010proBNP for OS [hazard ratio (HR) 1\u00b771; <jats:italic>P<\/jats:italic>\u00a0&lt;\u00a00\u00b7001]; gain 1q21 for haemEFS (HR 1\u00b768, <jats:italic>P<\/jats:italic>\u00a0=\u00a00\u00b7014), with a trend for OS (HR 1\u00b747, <jats:italic>P<\/jats:italic>\u00a0=\u00a00\u00b7084); difference between involved and uninvolved free light chains (dFLC) and light chain isotype for OS (HR 2\u00b722, <jats:italic>P<\/jats:italic>\u00a0&lt;\u00a00\u00b7001; HR 1\u00b762, <jats:italic>P<\/jats:italic>\u00a0=\u00a00\u00b7016) and haemEFS (HR 1\u00b788, <jats:italic>P<\/jats:italic>\u00a0&lt;\u00a00\u00b7001; HR 1\u00b759, <jats:italic>P<\/jats:italic>\u00a0=\u00a00\u00b7008). Estimated glomerular filtration rate (HR 0\u00b771, <jats:italic>P<\/jats:italic>\u00a0=\u00a00\u00b7004) and 24\u2010h proteinuria (HR 1\u00b710, <jats:italic>P<\/jats:italic>\u00a0=\u00a00\u00b7004) 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>","DOI":"10.1111\/bjh.17685","type":"journal-article","created":{"date-parts":[[2021,8,3]],"date-time":"2021-08-03T07:14:25Z","timestamp":1627974865000},"page":"230-243","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":21,"title":["Lenalidomide and dexamethasone in relapsed\/refractory immunoglobulin light chain (AL) amyloidosis: results from a\u00a0large cohort of patients with long follow\u2010up"],"prefix":"10.1111","volume":"195","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3829-0499","authenticated-orcid":false,"given":"Marco","family":"Basset","sequence":"first","affiliation":[{"name":"Division of Hematology\/Oncology Department of Internal Medicine V Heidelberg University Hospital Heidelberg Germany"},{"name":"Amyloidosis Center Heidelberg University Hospital Heidelberg Germany"},{"name":"Department of Molecular Medicine Amyloidosis Research and Treatment Center IRCCS Policlinico San Matteo University of Pavia Pavia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christoph R.","family":"Kimmich","sequence":"additional","affiliation":[{"name":"Division of Hematology\/Oncology Department of Internal Medicine V Heidelberg University Hospital Heidelberg Germany"},{"name":"Amyloidosis Center Heidelberg University Hospital Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicholas","family":"Schreck","sequence":"additional","affiliation":[{"name":"Division of Biostatistics German Cancer Research Center (DKFZ) Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julia","family":"Krzykalla","sequence":"additional","affiliation":[{"name":"Division of Biostatistics German Cancer Research Center (DKFZ) Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tobias","family":"Dittrich","sequence":"additional","affiliation":[{"name":"Division of Hematology\/Oncology Department of Internal Medicine V Heidelberg University Hospital Heidelberg Germany"},{"name":"Amyloidosis Center Heidelberg University Hospital Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kaya","family":"Veelken","sequence":"additional","affiliation":[{"name":"Division of Hematology\/Oncology Department of Internal Medicine V Heidelberg University Hospital Heidelberg Germany"},{"name":"Amyloidosis Center Heidelberg University Hospital Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hartmut","family":"Goldschmidt","sequence":"additional","affiliation":[{"name":"National Center for Tumor Diseases Heidelberg Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anja","family":"Seckinger","sequence":"additional","affiliation":[{"name":"Division of Hematology\/Oncology Department of Internal Medicine V Heidelberg University Hospital Heidelberg Germany"},{"name":"Laboratory of Hematology and Immunology &amp; Labor f\u00fcr Myelomforschung Vrije Universiteit Brussel (VUB) Jette Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dirk","family":"Hose","sequence":"additional","affiliation":[{"name":"Division of Hematology\/Oncology Department of Internal Medicine V Heidelberg University Hospital Heidelberg Germany"},{"name":"Laboratory of Hematology and Immunology &amp; Labor f\u00fcr Myelomforschung Vrije Universiteit Brussel (VUB) Jette Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"Jauch","sequence":"additional","affiliation":[{"name":"Institute of Human Genetics University Heidelberg Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carsten","family":"M\u00fcller\u2010Tidow","sequence":"additional","affiliation":[{"name":"Division of Hematology\/Oncology Department of Internal Medicine V Heidelberg University Hospital Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Axel","family":"Benner","sequence":"additional","affiliation":[{"name":"Division of Biostatistics German Cancer Research Center (DKFZ) Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ute","family":"Hegenbart","sequence":"additional","affiliation":[{"name":"Division of Hematology\/Oncology Department of Internal Medicine V Heidelberg University Hospital Heidelberg Germany"},{"name":"Amyloidosis Center Heidelberg University Hospital Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefan O.","family":"Sch\u00f6nland","sequence":"additional","affiliation":[{"name":"Division of Hematology\/Oncology Department of Internal Medicine V Heidelberg University Hospital Heidelberg Germany"},{"name":"Amyloidosis Center Heidelberg University Hospital Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2021,8,3]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41572-018-0034-3"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1159\/000507072"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2006-07-035352"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2006-07-032987"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2006-07-030544"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00277-011-1244-x"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.12973"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1080\/13506129.2018.1540410"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1093\/ndt\/gfq482"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2010-09-305136"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.3109\/13506129.2013.854766"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2014.57.4947"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2015-10-676361"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1038\/leu.2016.369"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2011-06-358507"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.20381"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2004.03.029"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2012-12-473066"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2014-04-570010"},{"key":"e_1_2_8_21_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.leu.2403586"},{"key":"e_1_2_8_22_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2011.37.7614"},{"key":"e_1_2_8_23_1","doi-asserted-by":"publisher","DOI":"10.1080\/13506129.2020.1868810"},{"key":"e_1_2_8_24_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2017-02-767467"},{"key":"e_1_2_8_25_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2017-02-767475"},{"key":"e_1_2_8_26_1","first-page":"1","article-title":"MICE: multivariate imputation by chained equations in R","author":"Buuren S","year":"2011","journal-title":"J. Stat. Softw."},{"key":"e_1_2_8_27_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41408-019-0266-9"},{"key":"e_1_2_8_28_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2016-11-751628"},{"key":"e_1_2_8_29_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2011.38.5724"},{"key":"e_1_2_8_30_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2013.50.8499"},{"key":"e_1_2_8_31_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.2020006913"},{"key":"e_1_2_8_32_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2016.163246"},{"key":"e_1_2_8_33_1","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.25775"},{"key":"e_1_2_8_34_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.2019003633"},{"key":"e_1_2_8_35_1","doi-asserted-by":"publisher","DOI":"10.3109\/13506129.2011.574354033"},{"key":"e_1_2_8_36_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2007-11-122101"},{"key":"e_1_2_8_37_1","doi-asserted-by":"publisher","DOI":"10.1080\/17474086.2020.1803060"},{"key":"e_1_2_8_38_1","doi-asserted-by":"crossref","unstructured":"DispenzieriA KastritisE WechalekarAD Sch\u00f6nland SO Kim K Sanchorawala V Landau HJ et\u00a0al.Primary results from the phase 3 tourmaline\u2010AL1 trial of ixazomib\u2010dexamethasone versus physician's choice of therapy in patients (Pts) with relapsed\/refractory primary systemic AL amyloidosis (RRAL). Blood. 2019;134(Suppl 1):139\u2013139.","DOI":"10.1182\/blood-2019-124409"},{"key":"e_1_2_8_39_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.16401"},{"key":"e_1_2_8_40_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.16471"},{"key":"e_1_2_8_41_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1516282"},{"key":"e_1_2_8_42_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1607751"},{"key":"e_1_2_8_43_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2018-05-850727"},{"key":"e_1_2_8_44_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.2019004369"},{"key":"e_1_2_8_45_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.2019004436"},{"key":"e_1_2_8_46_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.25828"},{"key":"e_1_2_8_47_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.26191"},{"key":"e_1_2_8_48_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clml.2018.08.021"},{"key":"e_1_2_8_49_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V124.21.4767.4767"},{"key":"e_1_2_8_50_1","doi-asserted-by":"publisher","DOI":"10.1182\/bloodadvances.2018023200"}],"container-title":["British Journal of Haematology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/bjh.17685","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/bjh.17685","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/bjh.17685","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,28]],"date-time":"2023-08-28T07:22:43Z","timestamp":1693207363000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/bjh.17685"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,8,3]]},"references-count":49,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2021,10]]}},"alternative-id":["10.1111\/bjh.17685"],"URL":"https:\/\/doi.org\/10.1111\/bjh.17685","archive":["Portico"],"relation":{},"ISSN":["0007-1048","1365-2141"],"issn-type":[{"value":"0007-1048","type":"print"},{"value":"1365-2141","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,8,3]]},"assertion":[{"value":"2021-03-24","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2021-06-15","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2021-08-03","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}