@article{3104526,
    title = "Real-world data on incidence, clinical characteristics and outcome of patients with macrofocal multiple myeloma (MFMM) in the era of novel therapies: A study of the Greco-Israeli collaborative myeloma working group",
    author = "Katodritou, E. and Kastritis, E. and Gatt, M. and Cohen, Y.C. and Avivi, I. and Pouli, A. and Lalayianni, C. and Lavi, N. and Delimpasis, S. and Kyrtsonis, M.-C. and Michael, M. and Suriu, C. and Miri, Z. and Tzafarti, K. and Vadikoliou, C. and Maltezas, D. and Zikos, P. and Ganzel, C. and Vaxman, Y. and Aviv, A. and Christoforidou, A. and Gavriatopoulou, M. and Shaulov, A. and Verrou, E. and Papanota, A.-M. and Fakinos, G. and Gkioka, A.-I. and Palaska, V. and Triantafyllou, T. and Konstantinidou, P. and Anagnostopoulos, A. and Terpos, E. and Dimopoulos, M.A.",
    journal = "American Journal of Hematology",
    year = "2020",
    volume = "95",
    number = "5",
    pages = "465-471",
    publisher = "Wiley-Liss, Inc.",
    issn = "0361-8609, 1096-8652",
    doi = "10.1002/ajh.25755",
    keywords = "bortezomib;  dexamethasone;  immunoglobulin;  lactate dehydrogenase;  lenalidomide;  proteasome inhibitor;  thalidomide, adult;  aged;  Article;  autologous stem cell transplantation;  bone disease;  cancer incidence;  cancer mortality;  cancer prognosis;  cancer radiotherapy;  cancer recurrence;  cancer staging;  clinical feature;  controlled study;  cytogenetics;  female;  follow up;  Greece;  human;  international cooperation;  Israel;  macrofocal multiple myeloma;  major clinical study;  male;  multiple myeloma;  multivariate analysis;  overall survival;  plasmacytoma;  prediction;  priority journal;  progression free survival;  retrospective study;  treatment outcome;  treatment response;  very elderly;  clinical trial;  data analysis;  incidence;  middle aged;  multicenter study;  multiple myeloma;  treatment outcome, Adult;  Aged;  Aged, 80 and over;  Data Analysis;  Female;  Greece;  Humans;  Incidence;  Israel;  Male;  Middle Aged;  Multiple Myeloma;  Retrospective Studies;  Treatment Outcome",
    abstract = "We investigated incidence, characteristics and outcome of patients with macrofocal multiple myeloma (MFMM) treated mainly with novel therapies. Based on definition (BMPCs <20% and lytic lesions/plasmacytomas, without anemia, renal insufficiency or hypercalcemia) we identified 140 patients with MFMM, among 4650 myeloma patients (3%). Twice the number of patients with typical myeloma were used as controls; 60% were <65 years and 70% had advanced bone disease. Plasmacytomas were more frequent in MFMM compared with standard myeloma (68% vs 15%, P <.05). Adverse prognostic parameters (high lactate dehydrogenase, advanced stage, high risk cytogenetics, immunoparesis) were less common in patients with MFMM compared with controls (P <.05); 90% received novel agents and 47% underwent autologous transplantation upfront; 90% achieved an objective response; 70% had at least very good partial response which was significantly higher compared with controls (P <.05). After a median follow-up of 52 months, 33 patients have died. Early death (<12 months) was infrequent in MFMM. Median progression-free survival and overall survival (OS) were 46 and 129 months respectively, both significantly longer compared with controls (P <.001). Proteasome inhibitor (PI)-based therapy was the only independent predictor for OS in the multivariate analysis (HR: 3.9; P <.001). In conclusion, MFMM is a distinct entity presented in young and elderly subjects, characterized by limited bone marrow infiltration, advanced bone disease and frequent presence of plasmacytomas; MFMM patients have less often adverse prognostic features and achieve excellent responses and prolonged OS especially when treated with PI-based therapies. Novel imaging will help in a more accurate classification of this entity. © 2020 Wiley Periodicals, Inc."
}