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Extramedullary Multiple Myeloma

Overview
Journal Leukemia
Specialties Hematology
Oncology
Date 2019 Nov 29
PMID 31776467
Citations 103
Authors
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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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References
1.
Blade J, Fernandez de Larrea C, Rosinol L, Cibeira M, Jimenez R, Powles R . Soft-tissue plasmacytomas in multiple myeloma: incidence, mechanisms of extramedullary spread, and treatment approach. J Clin Oncol. 2011; 29(28):3805-12. DOI: 10.1200/JCO.2011.34.9290. View

2.
Vande Broek I, Vanderkerken K, Van Camp B, Van Riet I . Extravasation and homing mechanisms in multiple myeloma. Clin Exp Metastasis. 2007; 25(4):325-34. DOI: 10.1007/s10585-007-9108-4. View

3.
Weinstock M, Ghobrial I . Extramedullary multiple myeloma. Leuk Lymphoma. 2012; 54(6):1135-41. DOI: 10.3109/10428194.2012.740562. View

4.
Fernandez de Larrea C, Kyle R, Durie B, Ludwig H, Usmani S, Vesole D . Plasma cell leukemia: consensus statement on diagnostic requirements, response criteria and treatment recommendations by the International Myeloma Working Group. Leukemia. 2013; 27(4):780-91. PMC: 4112539. DOI: 10.1038/leu.2012.336. View

5.
Touzeau C, Moreau P . How I treat extramedullary myeloma. Blood. 2015; 127(8):971-6. DOI: 10.1182/blood-2015-07-635383. View