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Immunoparesis defined by heavy plus light chain suppression is a novel marker of long-term outcomes in cardiac AL amyloidosis

Sachchithanantham, S; Berlanga, O; Alvi, A; Mahmood, SA; Lachmann, HJ; Gillmore, JD; Hawkins, PN; ... Wechalekar, AD; + view all (2017) Immunoparesis defined by heavy plus light chain suppression is a novel marker of long-term outcomes in cardiac AL amyloidosis. British Journal of Haematology , 179 (4) pp. 575-585. 10.1111/bjh.14908. Green open access

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Abstract

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 (≥2 Ig isotypes suppressed by >50% below normal levels) in 29 (17%) patients. Median overall survival (OS) on intention to treat (ITT) analysis was 26·2 months. In the ITT cohort, dFLC >180 mg/l was associated with shorter OS (P = 0·05); whereas HLC immunoparesis was not prognostic. On a landmark analysis of 127 patients alive at 6 months, presenting dFLC was not prognostic for OS (P = 0·33) and severe HLC immunoparesis trended towards poorer survival (20·2 vs. 42·8 months; P = 0·09). In the subset of patients with cardiac involvement, severe HLC immunoparesis conferred very poor outcome (median OS 8·8 vs. 29·9 months, P = 0·007). In conclusion, severe HLC immunoparesis is an independent marker of long-term poor prognosis in AL patients with cardiac involvement. The pathophysiological significance of this observation needs further study.

Type: Article
Title: Immunoparesis defined by heavy plus light chain suppression is a novel marker of long-term outcomes in cardiac AL amyloidosis
Open access status: An open access version is available from UCL Discovery
DOI: 10.1111/bjh.14908
Publisher version: http://dx.doi.org/10.1111/bjh.14908
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
Keywords: hevylite; immunoparesis; amyloid; cardiac; freelite
UCL classification: UCL
UCL > Provost and Vice Provost Offices
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine > Inflammation
URI: https://discovery.ucl.ac.uk/id/eprint/10043921
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