Mele Giuseppe, Pastore Domenico
Department of Haematology and BMT Unit, Antonio Perrino Hospital, Brindisi, Italy.
Case Rep Hematol. 2018 Nov 29;2018:2312430. doi: 10.1155/2018/2312430. eCollection 2018.
EMD of myeloma usually occurs several years after diagnosis and is associated with a very poor OS of <6 months due to the fact that there are no efficient treatment options. In rrMM with EMDs, the most effective treatment is a lymphoma-like polychemotherapy regimen such as PACE, Dexa-BEAM, and HyperCVAD followed by ASCT or allogeneic SCT. RT of soft-tissue plasmacytoma is the further treatment choice and results in a high rate of local control and a prolonged disease-free survival. We report the case of a 41-year-old man affected by ultra-high-risk symptomatic IgA MM with extramedullary intracranial soft-tissue relapsed after VTD-PACE followed by ASCT. The salvage program with KRd regimen determines a second biochemical and haematological remission and a gradual reduction in size of the extramedullary intracranial soft-tissue even in the absence of local aggressive radiotherapy, suggesting that carfilzomib and lenalidomide together could be effective also in this critical situation.
骨髓瘤的髓外疾病(EMD)通常在诊断后数年出现,由于缺乏有效的治疗选择,其总生存期(OS)非常短,不到6个月。在伴有EMD的复发/难治性多发性骨髓瘤(rrMM)中,最有效的治疗方法是采用类似淋巴瘤的多药化疗方案,如PACE、地塞米松-卡莫司汀、依托泊苷、阿糖胞苷和美法仑(Dexa-BEAM)以及高剂量环磷酰胺、长春新碱、阿霉素和地塞米松(HyperCVAD),随后进行自体造血干细胞移植(ASCT)或异基因造血干细胞移植(SCT)。软组织浆细胞瘤的放射治疗(RT)是进一步的治疗选择,可实现较高的局部控制率并延长无病生存期。我们报告了一例41岁男性患者的病例,该患者患有超高风险有症状的IgA型多发性骨髓瘤,在接受VTD-PACE方案及ASCT后出现髓外颅内软组织复发。采用卡非佐米、来那度胺和地塞米松(KRd)方案的挽救治疗实现了第二次生化和血液学缓解,即使在未进行局部强化放疗的情况下,髓外颅内软组织的大小也逐渐缩小,这表明卡非佐米和来那度胺联合使用在这种危急情况下也可能有效。