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

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. Adir Shaulovhttps://orcid.org/0000-0002-9620-781XEvangelos Terpos

Clinical characteristics of testicular extramedullary involvement in multiple myeloma

“…About 58% of the patients presented without BM involvement, thus emphasizing the value of novel imaging techniques especially in the relapsed setting. 8 Existing literature reports concomitant central nervous system (CNS) manifestations in a fifth of patients in the setting of EMD, 5 however this data was missing in our series, possibly due to lack of neurological symptoms warranting further investigation and future studies are needed to elucidate if routine CNS studies and chemotherapy prophylaxis is merited, as in high grade lymphomas. Role of local treatment like surgical orchiectomy and local radiation therapy outside of palliation of symptoms is largely unknown.…”

“…Interestingly, majority of patients with additional multiple PET defined FL had no concomitant BM involvement. 8 In the current series, 42.8% (6/14) had local treatment of the testicular EMD (five undergoing a surgical resection and one local radiation therapy) in addition to systemic therapy. Eleven patients were treated with multi-agent chemotherapy regimen (Table 1).…”

An Unusual Case of Multiple Myeloma with Light-Chain Cast Nephropathy Secondary to a Very Large Plasmacytoma without Bone Marrow Involvement

“…This presentation was similar to but also distinct from the rare phenomenon of macrofocal myeloma, in which multiple bone plasmacytomas are seen in the absence of bone marrow involvement. At this time, the standard of care for macrofocal multiple myeloma is systemic bortezomib-based therapy as a bridge to autologous stem cell transplant [ 16 , 17 ]. These patients typically respond well to treatment and have a significantly increased overall median survival compared to standard myeloma patients [ 17 ].…”

“…At this time, the standard of care for macrofocal multiple myeloma is systemic bortezomib-based therapy as a bridge to autologous stem cell transplant [ 16 , 17 ]. These patients typically respond well to treatment and have a significantly increased overall median survival compared to standard myeloma patients [ 17 ].…”

Clinical features of multiple myeloma with bone plasmacytomas

“…В исследовании E. Katodritou и соавт. доля плазматических клеток в пунктате костного мозга со ставила 14 %, в то время как среди пациентов без кост ных плазмоцитом в костном мозге обнаруживалось в среднем 63 % плазматических клеток [7]. В критери ях диагноза ММ, опубликованных Международной группой по изучению множественной миеломы (International Myeloma Working Group, IMWG) в 2014 г., установлено пороговое значение плазматических кле ток в костном мозге для верификации диагноза -10 %, однако авторы уточняют, что при множественных кост ных плазмоцитомах поражение костного мозга может быть неоднородным [8].…”