Central nervous system multiple myeloma: A real-world multi-institutional study of the Greek Myeloma Study Group

• Published in: American journal of hematology Vol. 99; no. 10; pp. 1897 - 1905
• Main Authors: Katodritou, Eirini, Dalampira, Dimitra, Delimpasi, Sosana, Ntanasis-Stathopoulos, Ioannis, Karaolidou, Fryni, Gkioka, Annita-Ioanna, Labropoulou, Vasiliki, Spanoudakis, Emmanouil, Triantafyllou, Theodora, Kotsopoulou, Maria, Michalis, Eyrydiki, Vadikolia, Chrysanthi, Papadopoulou, Theodosia, Sevastoudi, Aggeliki, Michael, Michael, Daiou, Aikaterini, Pentidou, Aikaterini, Kostopoulos, Ioannis, Palaska, Vasiliki, Gavriatopoulou, Maria, Kyrtsonis, Marie-Christine, Verrou, Evgenia, Kastritis, Efstathios, Dimopoulos, Meletios-Athanasios, Terpos, Evangelos
• Format: Journal Article
• Language: English
• Published: United States 02.07.2024
• ISSN: 0361-8609; 1096-8652; 1096-8652
• DOI: 10.1002/ajh.27425

Central nervous system (CNS) involvement is a rare and aggressive complication of multiple myeloma (MM). We identified 54/4352 MM patients (1.2%), who developed CNS-MM between 2000 and 2022. A matched-control group of MM patients without CNS-MM was used for comparisons. Median age was 63 years. Median time to CNS-MM was 28 months; 6/54 experienced CNS-MM at MM diagnosis. Abnormal lactate dehydrogenase (LDH), high-risk cytogenetics, and extramedullary involvement (EMI), that is, soft tissue plasmacytomas and/or plasma cell leukemia (PCL), were more frequent in CNS-MM versus controls (p < .05); 13/54 had PCL at CNS-MM. The majority had leptomeningeal infiltration (LMI) (66%); 26% had CNS-MM without systemic myeloma; EMI was the strongest predictor for CNS-MM (OR: 6.3). Median overall survival (OS) of CNS-MM patients versus controls was 43 months (95% CI: 32-54) versus 60 months (95% CI: 38-82) (p < .001); treatment of CNS-MM included mainly bortezomib/thalidomide/chemotherapy whereas 20% received novel drugs/immunotherapy combinations; 28 patients underwent cerebrospinal fluid infusions; EMI was the strongest negative predictor for post CNS-MM OS (p = .005; HR: 2.9). Treatment after 2016 predicted significantly for OS (p = .002; HR: 0.27). Median post CNS-MM OS was 4 months (95% CI: 2.6-5.4); in patients treated after 2016 median OS was 12 months. In conclusion, we have demonstrated in this large real-world series that survival of CNS-MM remains poor; however, there is a positive impact of treatment after 2016, related to the efficacy of modern anti-myeloma therapy; EMI significantly increases the probability to develop CNS-MM and the risk of post CNS-MM death, indicating a potential need for CNS prophylaxis for those patients.