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Clin Lymphoma Myeloma Leuk. 2018 Nov;18(11):e493-e499. doi: 10.1016/j.clml.2018.07.292. Epub 2018 Jul 25.

Outcome of Patients With Newly Diagnosed Systemic Light-Chain Amyloidosis Associated With Deletion of 17p.

Author information

1
Department of Medicine, Division of Hematology and Blood and Marrow Transplantation, University of California, San Francisco, CA.
2
Amyloidosis Center, University of Heidelberg, Heidelberg, Germany.
3
Amyloidosis Research and Treatment Center, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.
4
Memorial Sloan Kettering Cancer Center, New York, NY.
5
John C. Davis Myeloma and Amyloid Program, Tufts Medical Center, Boston, MA.
6
Department of Hematology, CHU Limoges, Centre National de Référence Maladies Rares, Limoges, France.
7
Hematology and Oncology Practice Altona (HOPA), Hamburg, Germany.
8
Amyloidosis and Myeloma Unit, Hospital Clínic de Barcelona, IDIBAPS, Barcelona, Spain.
9
Department of Clinical Therapeutics, Alexandra Hospital, National and Kapodistrian University of Athens, School of Medicine, Athens, Greece.
10
Division of Hematology, Mayo Clinic, Rochester, MN.
11
UK National Amyloidosis Centre, the Royal Free London NHS Foundation Trust, University College London, London, UK.
12
John C. Davis Myeloma and Amyloid Program, Tufts Medical Center, Boston, MA. Electronic address: rcomenzo@tuftsmedicalcenter.org.

Abstract

INTRODUCTION:

Deletion 17p (del 17p) portends a poor prognosis in myeloma, but its significance in light-chain amyloidosis is unknown.

PATIENTS AND METHODS:

We identified patients with light-chain amyloidosis and del 17p at diagnosis, and analyzed presenting characteristics, treatments, and clinical outcomes. All had baseline biopsy results showing amyloid and serologic and marrow studies, including standard fluorescence in-situ hybridization determinations of del 17p using commercial probes. Consensus criteria for hematologic and organ involvement, progression, and response were used. Kaplan-Meier (log rank) analyses and Cox regression analysis of baseline variables were used to identify predictors of overall and progression-free survival (PFS). Six-month landmark analyses were performed to assess the impact of treatment-related variables.

RESULTS:

We identified 44 patients from 7 countries with median marrow and del 17p plasma cells of 22% (range, 3%-100%) and 30% (2%-93%). Ninety-five percent had cardiac involvement, including 44% stage III. Two-thirds of the patients initially received bortezomib-based therapy. Forty-nine percent of patients experienced complete response or very good partial response, with median time to best response of 4 months (range, 1-28 months). Median overall survival and PFS were 49 and 32 months. Cardiac stage and hematologic response were the key predictors of outcomes. Patients with > 50% and ≤ 50% del 17p in clonal plasma cells had median survivals of 28 and 52 months, respectively (P = .08). In landmark analyses, only hematologic response predicted both overall survival and PFS.

CONCLUSION:

Cardiac stage, hematologic response, and del 17p percentage impact outcomes in these cases. Emphasis should be placed on optimizing supportive care and achieving a deep hematologic response.

KEYWORDS:

AL amyloidosis; Deletion 17p; FISH cytogenetics; Plasma cells; Prognosis

PMID:
30104177
DOI:
10.1016/j.clml.2018.07.292
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