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Multiple myeloma, gammopathies

Depth of organ response in AL amyloidosis is associated with improved survival: grading the organ response criteria

Leukemia volume 32, pages 2240–2249 (2018)Cite this article

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Abstract

Organ recovery following anti-plasma cell therapy is associated with improved outcome in patients with AL amyloidosis. Current organ response criteria do not consider the depth of response. Newly diagnosed AL amyloidosis patients who had involvement of at least one evaluable organ and documentation of organ response were included. Cardiac response was seen in 182 patients. Time to cardiac response was 9.4 months. Renal response was seen in 278 patients, at a median time to response of 6 months. Hepatic response was seen in 70 patients, with a median time to response of 6.1 months. In all organs, the deeper the organ response achieved, the longer the survival. This led to four organ response criteria: complete organ response (nadir NT-proBNP ≤400 pg/mL; nadir proteinuria ≤200 mg per 24 h; nadir alkaline phosphatase ≤×2 lower limit of normal); very good partial organ response (>60% reduction in parameter not meeting complete organ response definition); partial organ response (31–60% reduction in parameter); and non-responder (≤30% reduction in organ response parameter). Cardiac and renal progression were associated with baseline organ impairment and inversely associated with the depth of organ response. Grading the depth of organ response provides additional valuable prognostic information for newly diagnosed AL amyloidosis patients.

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Acknowledgements

The study was supported in part by the Jabbs Foundation (Birmingham, UK), the Henry J. Predolin Foundation (USA), and National Institutes of Health National Cancer Institute grant P50 CA186781.

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Authors and Affiliations

  1. Division of Hematology, Mayo Clinic, Rochester, MN, USA

    Eli Muchtar, Angela Dispenzieri, Nelson Leung, Martha Q. Lacy, Francis K. Buadi, David Dingli, Suzanne R. Hayman, Prashant Kapoor, Yi Lisa Hwa, Amie Fonder, Miriam Hobbs, Rajshekhar Chakraborty, Wilson Gonsalves, Taxiarchis V. Kourelis, Rahma Warsame, Stephen Russell, John A. Lust, Yi Lin, Ronald S. Go, Steven Zeldenrust, Robert A. Kyle, S. Vincent Rajkumar, Shaji K. Kumar & Morie A. Gertz

  2. Division of Nephrology and Hypertension, Mayo Clinic, Rochester, MN, USA

    Nelson Leung

  3. Department of Cardiovascular Diseases, Mayo Clinic, Rochester, MN, USA

    Martha Grogan

  4. Hospitalist Services, Essentia Health St. Joseph’s Hospital, Brainerd, MN, USA

    Rajshekhar Chakraborty

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  1. Eli Muchtar
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Correspondence to Morie A. Gertz.

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Conflict of interest

A.D. received research funding from Celgene, Millennium, Pfizer, and Janssen and travel grant from Pfizer. M.Q.L. received research funding from Celgene. D.D. received research funding from Karyopharm Therapeutics, Amgen, and Millenium Pharmaceuticals. P.K. received research funding from Takeda, Celgene, and Amgen. S.K.K. received consultancy from Celgene, Millennium, Onyx, Janssen, and BMS, and research funding from Celgene, Millennium, Novartis, Onyx AbbVie, Janssen, and BMS. M.A.G. received consultancy from Millenium and honoraria from Celgene, Millenium, Onyx, Novartis, Smith Kline, Prothena, Ionis, and Amgen. The remaining authors declare that they have no conflict of interest.

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Muchtar, E., Dispenzieri, A., Leung, N. et al. Depth of organ response in AL amyloidosis is associated with improved survival: grading the organ response criteria. Leukemia 32, 2240–2249 (2018). https://doi.org/10.1038/s41375-018-0060-x

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