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Current Hematologic Malignancy Reports
Published in: Current Hematologic Malignancy Reports 2/2020

01-04-2020 | Multiple Myeloma | Multiple Myelomas (P Kapoor, Section Editor)

Extramedullary Disease in Multiple Myeloma

Authors: Megan H. Jagosky, Saad Z. Usmani

Published in: Current Hematologic Malignancy Reports | Issue 2/2020

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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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Metadata
Title
Extramedullary Disease in Multiple Myeloma
Authors
Megan H. Jagosky
Saad Z. Usmani
Publication date
01-04-2020
Publisher
Springer US
Published in
Current Hematologic Malignancy Reports / Issue 2/2020
Print ISSN: 1558-8211
Electronic ISSN: 1558-822X
DOI
https://doi.org/10.1007/s11899-020-00568-3

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