{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,27]],"date-time":"2026-05-27T13:45:56Z","timestamp":1779889556340,"version":"3.53.1"},"reference-count":42,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2017,10,8]],"date-time":"2017-10-08T00:00:00Z","timestamp":1507420800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Br J Haematol"],"published-print":{"date-parts":[[2017,11]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Cardiac involvement and presenting dFLC (difference between involved and uninvolved free light chains) are independent predictors of outcome in systemic AL amyloidosis. These markers have limited prognostic utility in patients surviving the initial months following diagnosis. Here we assessed immunoparesis, as determined by novel heavy+light chain (HLC) immunoassays, as a prognostic marker for survival in AL amyloidosis. HLC measurements identified immunoparesis of at least one immunoglobulin (Ig) isotype in 145 (85%) patients; and severe immunoparesis (\u22652 Ig isotypes suppressed by &gt;50% below normal levels) in 29 (17%) patients. Median overall survival (OS) on intention to treat (ITT) analysis was 26\u00b72\u00a0months. In the ITT cohort, dFLC &gt;180\u00a0mg\/l was associated with shorter OS (<jats:italic>P<\/jats:italic>\u00a0=\u00a00\u00b705); whereas HLC immunoparesis was not prognostic. On a landmark analysis of 127 patients alive at 6\u00a0months, presenting dFLC was not prognostic for OS (<jats:italic>P<\/jats:italic>\u00a0=\u00a00\u00b733) and severe HLC immunoparesis trended towards poorer survival (20\u00b72 vs. 42\u00b78\u00a0months; <jats:italic>P<\/jats:italic>\u00a0=\u00a00\u00b709). In the subset of patients with cardiac involvement, severe HLC immunoparesis conferred very poor outcome (median OS 8\u00b78 vs. 29\u00b79\u00a0months, <jats:italic>P<\/jats:italic>\u00a0=\u00a00\u00b7007). In conclusion, severe HLC immunoparesis is an independent marker of long\u2010term poor prognosis in AL patients with cardiac involvement. The pathophysiological significance of this observation needs further study.<\/jats:p>","DOI":"10.1111\/bjh.14908","type":"journal-article","created":{"date-parts":[[2017,10,9]],"date-time":"2017-10-09T03:16:39Z","timestamp":1507518999000},"page":"575-585","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":18,"title":["Immunoparesis defined by heavy+light chain suppression is a novel marker of long\u2010term outcomes in cardiac AL amyloidosis"],"prefix":"10.1111","volume":"179","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2389-1606","authenticated-orcid":false,"given":"Sajitha","family":"Sachchithanantham","sequence":"first","affiliation":[{"name":"Centre for Amyloidosis and Acute Phase Proteins University College London (Royal Free Campus) London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4844-6031","authenticated-orcid":false,"given":"Oscar","family":"Berlanga","sequence":"additional","affiliation":[{"name":"The Binding Site Group Ltd. Birmingham UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Azra","family":"Alvi","sequence":"additional","affiliation":[{"name":"The Binding Site Group Ltd. Birmingham UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shameem A.","family":"Mahmood","sequence":"additional","affiliation":[{"name":"Centre for Amyloidosis and Acute Phase Proteins University College London (Royal Free Campus) London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Helen J.","family":"Lachmann","sequence":"additional","affiliation":[{"name":"Centre for Amyloidosis and Acute Phase Proteins University College London (Royal Free Campus) London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julian D.","family":"Gillmore","sequence":"additional","affiliation":[{"name":"Centre for Amyloidosis and Acute Phase Proteins University College London (Royal Free Campus) London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philip N.","family":"Hawkins","sequence":"additional","affiliation":[{"name":"Centre for Amyloidosis and Acute Phase Proteins University College London (Royal Free Campus) London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephen","family":"Harding","sequence":"additional","affiliation":[{"name":"The Binding Site Group Ltd. Birmingham UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ashutosh D.","family":"Wechalekar","sequence":"additional","affiliation":[{"name":"Centre for Amyloidosis and Acute Phase Proteins University College London (Royal Free Campus) London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2017,10,8]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"crossref","first-page":"1818a","DOI":"10.1182\/blood.V114.22.1818.1818","article-title":"Heavy\/light chain specific immunoglobulin ratios at presentation are prognostic for progression free survival in the IFM 2005\u201001 myeloma trial","volume":"114","author":"Avet\u2010Loiseau H.","year":"2009","journal-title":"Blood"},{"key":"e_1_2_7_3_1","first-page":"P\u2010382a","article-title":"Multiple myeloma outcome is associated with polyclonal immunoglobulin suppression of the same isotype as the tumour","volume":"96","author":"Avet\u2010Loiseau 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