Abstract
Immunoparesis is an adverse prognostic marker in plasma cell proliferative disorders. Its impact in AL amyloidosis has not been explored in depth. Newly diagnosed AL amyloidosis patients (n=998) were evaluated for immunoparesis by two methods. The first method was qualitative, considering the number of suppressed uninvolved immunoglobulins below the lower limit of normal (LLN) (none, partial, all). The second method was quantitative, assessing the average relative difference (ARD) of the uninvolved immunoglobulins from the LLN. Patients with suppression of all the uninvolved immunoglobulins were less likely to achieve very good partial response (VGPR) or better to first-line treatment (44%) compared with patients with partial suppression (68%) or preserved uninvolved immunoglobulins (64%; P<0.0001). In addition, patients with suppression of all the uninvolved immunoglobulins had a shorter survival compared with the respective comparators (median 18 vs 54 vs 52 months; P<0.0001). In the quantitative method, patients with a negative ARD were less likely to achieve VGPR or better (48%) and had a shorter survival (median 24 months) compared with patients with a positive ARD (69%, 57 months, respectively; P<0.0001). In a multivariate analysis for survival, both assessment methods retained an independent impact. Significant immunoparesis has a negative impact on response and survival in newly diagnosed AL amyloidosis.
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Acknowledgements
We would like to thank two patients, UN and PG, for inspiring the idea of the role of immunoparesis in AL amyloidosis
Author contributions
EM designed the study, analyzed the data, wrote the first draft and approved the final version of the manuscript; AD, SKK, FKB, MQL, SZ, SRH, NL, TVK, WG, SR, DD, JAL, YL, PK, RG and VSK performed patient management, revised the manuscript critically and participated in final data analysis and approval of the final version of the manuscript. RC analyzed data, provided critical review of the manuscript and approved the last version of the manuscript; RAK performed patients’ follow-up, revised the manuscript critically and participated in final data analysis and approval of the final version of the manuscript; MAG performed patient management, designed the study, analyzed the data, wrote the first draft and approved the final version of the manuscript.
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Muchtar, E., Dispenzieri, A., Kumar, S. et al. Immunoparesis in newly diagnosed AL amyloidosis is a marker for response and survival. Leukemia 31, 92–99 (2017). https://doi.org/10.1038/leu.2016.140
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DOI: https://doi.org/10.1038/leu.2016.140
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