RESUMO
We retrospectively analyzed the clinical features and outcomes in a real-world cohort of adolescents and the young adult (AYA) patients (age between 16 and 39 years) with symptomatic multiple myeloma (MM) registered with the Kansai Myeloma Forum. 26 patients had been diagnosed as symptomatic MM out of 3284 patients. The prevalence of AYA-MM was 0.8% in this cohort. 81% of the patients was received stem cell transplantation, which may improve outcome. Anemia and hypercalcemia might be prognostic factors, however International Staging System failed to predict overall survival. Five patients developed late-onset adverse events which were serious and life-threatening. The 5-year overall survival was 71.0%. We need to develop the new strategy to overcome AYA-MM.
Assuntos
Mieloma Múltiplo/terapia , Sistema de Registros , Transplante de Células-Tronco , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Anemia , Estudos de Coortes , Feminino , Humanos , Hipercalcemia , Japão , Masculino , Pessoa de Meia-Idade , Mieloma Múltiplo/epidemiologia , Mieloma Múltiplo/mortalidade , Prognóstico , Estudos Retrospectivos , Transplante de Células-Tronco/efeitos adversos , Taxa de Sobrevida , Resultado do Tratamento , Adulto JovemRESUMO
We retrospectively analyzed 51 patients with solitary plasmacytoma diagnosed from October 2002 to September 2018 from a cohort of 3575 patients with plasma cell dyscrasias registered in the Kansai Myeloma Forum. Twenty-seven patients had solitary bone plasmacytoma (SBP) and 24 had extramedullary plasmacytoma (EMP), with prevalence of 0.8% and 0.7%, respectively. The most frequent M protein was IgG (40%) in SBP, whereas non-secretory proteins were most frequent (50%) in EMP. Five-year overall survival was 78.2% in SBP and 80.8% in EMP (P = 0.894). Among patients with SBP, 44% progressed to MM with a median time of 10.5 months (2.4-93.3 months), whereas 8% of EMP patients progressed to MM with a median time of 18.6 months (13.0-24.2 months). The most frequent treatment was radiotherapy (41%) or observation (41%) in SBP, and chemotherapy (54%) in EMP. No statistically significant difference was observed upon univariate analysis of prognostic factors including age, sex, performance status, and IgG M protein. Our results suggest that there are biological differences between SBP and EMP in real-world settings.
Assuntos
Neoplasias Ósseas , Plasmocitoma , Sistema de Registros , Adulto , Idoso , Idoso de 80 Anos ou mais , Neoplasias Ósseas/epidemiologia , Neoplasias Ósseas/mortalidade , Neoplasias Ósseas/patologia , Neoplasias Ósseas/terapia , Progressão da Doença , Feminino , Humanos , Imunoglobulina G , Japão/epidemiologia , Masculino , Pessoa de Meia-Idade , Mieloma Múltiplo/etiologia , Proteínas do Mieloma , Plasmocitoma/epidemiologia , Plasmocitoma/mortalidade , Plasmocitoma/patologia , Plasmocitoma/terapia , Prognóstico , Estudos Retrospectivos , Taxa de SobrevidaRESUMO
We retrospectively analyzed twenty-six patients with primary plasma cell leukemia (pPCL) registered from May 2005 until April 2015 by the Kansai Myeloma Forum. Twenty patients received novel agents (bortezomib or lenalidomide), and their median survival of was 34 months. The median survival of patients who underwent autologous stem cell transplantation (SCT) was 40 months, those undergoing allogeneic SCT 55 months, and those undergoing both types of SCT (auto-allo) 61 months; whereas for those who did not undergo SCT it was 28 months (pâ¯=â¯0.845). The only statistically significant risk factor identified by multivariate analysis was hypercalcemia.