Extramedullary Disease in Multiple Myeloma

Curr Hematol Malig Rep. 2020 Apr;15(2):62-71. doi: 10.1007/s11899-020-00568-3.


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

Keywords: Extramedullary; Immunomodulatory drugs; Multiple myeloma; Plasma cell leukemia; Proteasome inhibitor; Transplant.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Biomarkers, Tumor / genetics
  • Cell Proliferation* / drug effects
  • Gene Deletion
  • Genetic Predisposition to Disease
  • Humans
  • Multiple Myeloma / genetics
  • Multiple Myeloma / mortality
  • Multiple Myeloma / pathology*
  • Multiple Myeloma / therapy
  • Phenotype
  • Plasma Cells / drug effects
  • Plasma Cells / pathology*
  • Risk Factors
  • Stem Cell Transplantation
  • Translocation, Genetic
  • Treatment Outcome


  • Biomarkers, Tumor