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.