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Multiple myeloma gammopathies

Extramedullary multiple myeloma

Leukemia volume 34, pages 1–20 (2020)Cite this article

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Abstract

Extramedullary multiple myeloma (EMM) is an aggressive subentity of multiple myeloma, characterized by the ability of a subclone to thrive and grow independent of the bone marrow microenvironment, resulting in a high-risk state associated with increased proliferation, evasion of apoptosis and treatment resistance. Despite improvement in survival for most patients with multiple myeloma over recent decades, outcomes are generally poor when EMM develops. Understanding the molecular underpinnings leading to homing of plasma cells in ecosystems outside the bone marrow will be crucial for therapeutically manipulating the microenvironment and targeting key signaling pathways. Herein, we discuss the evolutionary biology of EMM, underscore the importance of a uniform definition, discuss prognostic significance, and provide current and emerging treatment strategies for managing this rare subentity of multiple myeloma.

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Acknowledgements

This work is supported by the Carolinas Myeloma Research Fund, Heinemann Foundation of Charlotte, the Freedland Fund, the Leukemia Lymphoma Society and NCI Grant 5R01CA201634.

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Authors and Affiliations

  1. Department of Hematologic Oncology and Blood Disorders, Levine Cancer Institute/Atrium Health, Charlotte, NC, USA

    Manisha Bhutani, Shebli Atrash, Peter M. Voorhees & Saad Z. Usmani

  2. Immune Monitoring Core Laboratory, Levine Cancer Institute/Atrium Health, Charlotte, NC, USA

    David M. Foureau

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  1. Manisha Bhutani
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  2. David M. Foureau
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  4. Peter M. Voorhees
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Contributions

SZU and MB conceived and designed the study. MB wrote the first draft of the manuscript with input from SZU and PMV. DMF, MB and SA prepared the figures. All authors were involved in researching data, discussion of content and critical revision of the paper and approval of the submitted manuscript.

Corresponding author

Correspondence to Saad Z. Usmani.

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Conflict of interest

MB has received speaker’s fees from Amgen. DMF has no conflict of interest. SA has received consulting fees from Takeda, Amgen and Celgene, and speaker’s fees from Takeda and Celgene. PMV has received consulting fees from Amgen, BMS, Celgene, Janssen, Novartis, Oncopeptides, Takeda and TeneoBio. SZU has received consulting fees from Abbvie, Amgen, BMS, Celgene, EdoPharma, GSK, Janssen, Sanofi, Seattle Genetics, Skyline Dx, Takeda and TeneoBio, and research funding from Amgen, Array Biopharma, BMS, Celgene, Janssen, Pharmacyclics, Prothena, Sanofi, Seattle Genetics, Skyline Dx and Takeda.

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Bhutani, M., Foureau, D.M., Atrash, S. et al. Extramedullary multiple myeloma. Leukemia 34, 1–20 (2020). https://doi.org/10.1038/s41375-019-0660-0

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