@article{0b2f573141e2442d946c20a76e784457,
title = "Characterization and prognostic implication of delayed complete response in AL amyloidosis",
abstract = "Introduction: Little is known on continued response following completion of therapy in light chain (AL) amyloidosis. Methods: We studied 373 AL amyloidosis patients who achieved complete response (CR) or very good partial response (VGPR) to first-line therapy. Results: By end of therapy (EOT), 46% of patients achieved a CR and 54% a VGPR. With no further therapy, 17.5% of patients were upstaged from VGPR to CR (delayed CR), with a median of 9 months. Compared with CR and VGPR at EOT, patients with a delayed CR were characterized by higher proportion of t(11;14) and lower rate of trisomies. Autologous stem cell transplant was more frequent in the delayed CR group. Patients with a delayed CR were characterized by minimal residual disease negativity and organ response rates similar to patients with CR at EOT and higher than patients achieving VGPR at EOT. Patients with a delayed CR had a longer PFS/OS compared to patients with CR or VGPR by EOT (median PFS 149 vs 92 vs 52 months, P <.001; 10-year OS 87% vs 71% vs 56%, P <.001). Conclusions: This study characterizes delayed CR in AL amyloidosis, highlights its prognostic impact which is at least similar to those who achieved CR at EOT, and underlines another aspect of response monitoring.",
keywords = "autologous stem cell transplant, immunoparesis, off-therapy, survival",
author = "Eli Muchtar and Gertz, {Morie A.} and Kumar, {Shaji K.} and Lacy, {Martha Q.} and Nelson Leung and Buadi, {Francis K.} and David Dingli and Hayman, {Suzanne R.} and Go, {Ronald S.} and Prashant Kapoor and Wilson Gonsalves and Kourelis, {Taxiarchis V.} and Rahma Warsame and {Lisa Hwa}, Yi and Amie Fonder and Miriam Hobbs and Stephen Russell and Lust, {John A.} and Mustaqueem Siddiqui and {Vincent Rajkumar}, S. and Kyle, {Robert A.} and Angela Dispenzieri",
note = "Funding Information: The study was supported in part by the Jabbs Foundation (Birmingham, United Kingdom), the Henry J. Predolin Foundation (USA), and National Institutes of Health National Cancer Institute grant P50 CA186781. Funding Information: Morie A. Gertz received consultancy from Millennium and honoraria from Celgene, Millennium, Onyx, Novartis, Smith Kline, Prothena, and Ionis. Shaji Kumar received consultancy from Celgene, Millennium, Onyx, Janssen, and BMS; and research funding from Celgene, Millennium, Novartis, Onyx AbbVie, Janssen, and BMS. Martha Q. Lacy received research funding from Celgene; David Dingli received research funding from Karyopharm Therapeutics, Amgen, and Millennium Pharmaceuticals; Prashant Kapoor received research funding from Takeda, Celgene, and Amgen; Angela Dispenzieri received research funding from Celgene, Millennium, Pfizer, and Alnylam, travel grant from Pfizer, and advisory board from Janssen. Eli Muchtar, Nelson Leung, Francis Buadi, Suzanne R. Hayman, Ronald S. Go, Wilson Gonsalves, Taxiarchis v. Kourelis, Rahma Warsame, Yi Lisa Hwa, Amie Fonder, Miriam Hobbs, Stephen Russel, John A. Lust, Mustaqueem Siddiqui, Vincent Rajkumar, and Robert A. Kyle have no conflict of interest. Publisher Copyright: {\textcopyright} 2020 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd",
year = "2021",
month = mar,
doi = "10.1111/ejh.13554",
language = "English (US)",
volume = "106",
pages = "354--361",
journal = "European Journal of Haematology",
issn = "0902-4441",
publisher = "Wiley-Blackwell",
number = "3",
}