Megan H Jagosky1, Saad Z Usmani2. 1. Plasma Cell Disorders, Department of Hematologic Oncology & Blood Disorders, Levine Cancer Institute/Atrium Health UNC School of Medicine, Charlotte, NC, USA. 2. Plasma Cell Disorders, Department of Hematologic Oncology & Blood Disorders, Levine Cancer Institute/Atrium Health UNC School of Medicine, Charlotte, NC, USA. saad.usmani@atriumhealth.org.
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. The current review summarizes the disease characterization data, along with available data on clinical activity of available anti-MM agents for this entity.
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 MMpatients, 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.
Authors: Joan Bladé; Meral Beksac; Jo Caers; Artur Jurczyszyn; Marie von Lilienfeld-Toal; Philippe Moreau; Leo Rasche; Laura Rosiñol; Saad Z Usmani; Elena Zamagni; Paul Richardson Journal: Blood Cancer J Date: 2022-03-21 Impact factor: 9.812
Authors: Eva Kriegova; Regina Fillerova; Jiri Minarik; Jakub Savara; Jirina Manakova; Anna Petrackova; Martin Dihel; Jana Balcarkova; Petra Krhovska; Tomas Pika; Petr Gajdos; Marek Behalek; Michal Vasinek; Tomas Papajik Journal: Sci Rep Date: 2021-07-19 Impact factor: 4.379
Authors: Sagar Lonial; Hans C Lee; Ashraf Badros; Suzanne Trudel; Ajay K Nooka; Ajai Chari; Al-Ola Abdallah; Natalie Callander; Douglas Sborov; Attaya Suvannasankha; Katja Weisel; Peter M Voorhees; Lynsey Womersley; January Baron; Trisha Piontek; Eric Lewis; Joanna Opalinska; Ira Gupta; Adam D Cohen Journal: Cancer Date: 2021-07-27 Impact factor: 6.860