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Amyloid
The Journal of Protein Folding Disorders
Volume 25, 2018 - Issue 2
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Original Article

Bone marrow plasma cell infiltration in light chain amyloidosis: impact on organ involvement and outcome

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Pages 79-85 | Received 07 Dec 2017, Accepted 18 Feb 2018, Published online: 26 Feb 2018
 

Abstract

Objectives: Prognosis of immunoglobulin light-chain (AL) amyloidosis depends mainly on the presence of cardiac involvement and the disease burden. A higher bone marrow plasma cell (BMPC) burden has been recognized as an adverse prognostic factor. The aim of our study was to analyze the correlation between the BMPC infiltration, clinical features and outcomes in patients with AL amyloidosis.

Methods: The clinical records of 79 patients with AL amyloidosis treated at a single institution.

Results: Median BMPC infiltration at diagnosis was 11% and significantly correlated with the serum free light-chain difference (p < .001). Patients with more than 10% BMPCs had more frequent cardiac involvement (86 vs. 63%; p = .015), a trend towards a higher early mortality (27 vs. 11%; p = .08) and a significantly shorter progression-free survival (PFS) (median of 18 vs. 48 months, p = .02) and overall survival (median of 33 months vs. not reached; p = .046). In the multivariate analysis, a BMPC infiltration over 10% retained its adverse prognostic value for PFS (HR = 2.26; 95% CI, 1.048–4.866; p = .038). The use of new drugs seemed to overcome the negative prognostic impact of a higher BMPC infiltration.

Conclusion: Higher BMPC infiltration in AL amyloidosis might be associated with increased systemic organ damage, particularly cardiac involvement and is rarely related to the development of myeloma features.

Disclosure statement

No potential conflict of interest was reported by the authors.

Additional information

Funding

This work has been supported in part by grants [FIS PI16/00423] and [PI12/01290] from Instituto de Salud Carlos III and Fondo Europeo de Desarrollo Regional (FEDER) and 2014SGR-552 from AGAUR (Generalitat de Catalunya).

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