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Extramedullary Disease in Multiple Myeloma

  • Multiple Myelomas (P Kapoor, Section Editor)
  • Published:
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Abstract

Purpose of Review

Extramedullary disease (EMD) is a rare but recognized manifestation of multiple myeloma (MM), characterized by involvement of several organs including skin, liver, lymphatic system, pleura, and central nervous system. The incidence is about 3–5% in newly diagnosed MM patients, but has been reported in up to 20% patients in the relapsed MM setting.

Recent Findings

Presence of EMD has been associated with more aggressive phenotype of MM, elevated serum lactate dehydrogenase (LDH) enzyme, and high-risk cytogenetics [deletion 17p, translocation (4;14), translocation (14;16)]. There are several hypotheses of how EMD occurs, including factors leading to bone marrow emancipation and hematogenous spread. The treatment schema usually follows that of high-risk MM.

Summary

The current review summarizes the disease characterization data, along with available data on clinical activity of available anti-MM agents for this entity.

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Funding

Saad Z. Usmani is supported by the Carolinas Myeloma Research Fund, Heinemann Foundation of Charlotte, the Freedland Fund, and the Leukemia Lymphoma Society.

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Authors and Affiliations

  1. Plasma Cell Disorders, Department of Hematologic Oncology & Blood Disorders, Levine Cancer Institute/Atrium Health UNC School of Medicine, Charlotte, NC, USA

    Megan H. Jagosky & Saad Z. Usmani

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  1. Megan H. Jagosky
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Correspondence to Saad Z. Usmani.

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Conflict of Interest

Megan H. Jagosky declares that she has no conflict of interest.

Saad Z. Usmani has received consulting fees from Abbvie, Amgen, BMS, Celgene, EdoPharma, GSK, Janssen, Sanofi, Seattle Genetics, SkylineDx, Takeda, and TeneoBio; and research funding from Amgen, Array Biopharma, BMS, Celgene, Janssen, Pharmacyclics, Prothena, Sanofi, Seattle Genetics, SkylineDx, and Takeda.

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Jagosky, M.H., Usmani, S.Z. Extramedullary Disease in Multiple Myeloma. Curr Hematol Malig Rep 15, 62–71 (2020). https://doi.org/10.1007/s11899-020-00568-3

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