{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,6]],"date-time":"2026-05-06T17:15:27Z","timestamp":1778087727261,"version":"3.51.4"},"reference-count":19,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2026,2,22]],"date-time":"2026-02-22T00:00:00Z","timestamp":1771718400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,2,22]],"date-time":"2026-02-22T00:00:00Z","timestamp":1771718400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Internal Medicine Journal"],"published-print":{"date-parts":[[2026,4]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Immunoglobulin light chain (AL) amyloidosis is a rare but devastating monoclonal gammopathy where organ failure develops secondary to tissue deposition of misfolded immunoglobulin light chains.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Aims<\/jats:title>\n                    <jats:p>We aimed to describe the characteristics and outcomes of a uniformly treated cohort of Australian patients with AL amyloidosis.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Subjects required a histological diagnosis of AL amyloidosis, symptomatic organ involvement and initial treatment with a bortezomib\u2010based regimen. Kaplan\u2013Meier survival analysis was performed and the impact of baseline variables on response was assessed using Cox regression testing.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Of 271 patients with AL amyloidosis, median age was 66\u2009years and 53% were male. Seventy\u2010five per cent of cases were lambda restricted, 79% had cardiac involvement and 64% had renal involvement. Overall haematological response rate was 82% being complete in 31% and very good partial response in 36%. Eight per cent of patients died prior to response assessment. Median overall survival was 74 months with inferior overall survival independently associated with more advanced cardiac disease (\n                      <jats:italic>P<\/jats:italic>\n                      =\u20090.022 for cardiac stage IIIA and\n                      <jats:italic>P<\/jats:italic>\n                      &lt;\u20090.0001 for stage IIIB), age (\n                      <jats:italic>P<\/jats:italic>\n                      =\u20090.016) and autonomic neuropathy (\n                      <jats:italic>P<\/jats:italic>\n                      =\u20090.003). A 6\u2010month landmark analysis demonstrated a significant impact of the depth of haematologic response on overall survival.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>We provide the first description of a large cohort of Australian patients with AL amyloidosis. Two\u2010thirds of patients achieve deep haematological responses with bortezomib\u2010based therapy. We confirm the major impact of the severity of cardiac involvement on survival in AL amyloidosis, with stage IIIB disease portending a particularly poor outcome.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/imj.70335","type":"journal-article","created":{"date-parts":[[2026,2,23]],"date-time":"2026-02-23T03:35:51Z","timestamp":1771817751000},"page":"582-588","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["An\u00a0Australian Amyloidosis Network analysis of Australian patients with immunoglobulin light chain amyloidosis treated with bortezomib\u2010based chemotherapy"],"prefix":"10.1111","volume":"56","author":[{"ORCID":"https:\/\/orcid.org\/0009-0006-0869-2293","authenticated-orcid":false,"given":"Anthea","family":"Gibbons","sequence":"first","affiliation":[{"name":"Princess Alexandra Hospital  Brisbane Queensland Australia"},{"name":"School of Medicine University of Queensland  Brisbane 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Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fiona","family":"Kwok","sequence":"additional","affiliation":[{"name":"Westmead Hospital  Sydney New South Wales Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6919-7062","authenticated-orcid":false,"given":"Brendan","family":"Wisniowski","sequence":"additional","affiliation":[{"name":"Eastern Health  Melbourne Victoria Australia"},{"name":"Eastern Clinical School Monash University  Melbourne Victoria Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6684-1000","authenticated-orcid":false,"given":"Nirija","family":"Ranjit Anderson","sequence":"additional","affiliation":[{"name":"Princess Alexandra Hospital  Brisbane Queensland Australia"},{"name":"School of Medicine University of Queensland  Brisbane 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