TY - JOUR
TI - 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
AU - Katodritou, E.
AU - Kastritis, E.
AU - Gatt, M.
AU - Cohen, Y.C.
AU - Avivi, I.
AU - Pouli, A.
AU - Lalayianni, C.
AU - Lavi, N.
AU - Delimpasis, S.
AU - Kyrtsonis, M.-C.
AU - Michael, M.
AU - Suriu, C.
AU - Miri, Z.
AU - Tzafarti, K.
AU - Vadikoliou, C.
AU - Maltezas, D.
AU - Zikos, P.
AU - Ganzel, C.
AU - Vaxman, Y.
AU - Aviv, A.
AU - Christoforidou, A.
AU - Gavriatopoulou, M.
AU - Shaulov, A.
AU - Verrou, E.
AU - Papanota, A.-M.
AU - Fakinos, G.
AU - Gkioka, A.-I.
AU - Palaska, V.
AU - Triantafyllou, T.
AU - Konstantinidou, P.
AU - Anagnostopoulos, A.
AU - Terpos, E.
AU - Dimopoulos, M.A.
JO - American Journal of Hematology
PY - 2020
VL - 95
TODO - 5
SP - 465-471
PB - Wiley-Liss, Inc.
SN - 0361-8609, 1096-8652
TODO - 10.1002/ajh.25755
TODO - 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
TODO - 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.
ER -