Jamison Lee S, Mo Clifton Craig, Kwok Mary
Department of Internal Medicine, Fort Belvoir Community Hospital, Fort Belvoir, Virginia, USA
Department of Medical Oncology, Dana Farber Cancer Institute, Boston, Massachusetts, USA.
BMJ Case Rep. 2020 Apr 23;13(4):e233340. doi: 10.1136/bcr-2019-233340.
In patients who experience relapse of multiple myeloma, upwards of 30% can have extramedullary disease. The presence of extramedullary multiple myeloma is typically associated with adverse cytogenetics and a poor prognosis. Organs most commonly involved include the liver, skin, central nervous system, pleural effusions, kidney, lymph nodes, and pancreas. We present the case of a 53-year-old man with IgA kappa multiple myeloma with the adverse cytogenetic findings of t(4;14) and 1q21 gain who had achieved a stringent complete (sCR) response after initial therapy with carfilzomib, lenalidomide and dexamethasone. Stringent complete response is defined as the normalization of the free light chain ratio in the serum and an absence of clonal cells in the bone marrow in additiion to criteria needed to achieve complete response. Prior to undergoing a planned autologous stem cell transplant, this patient experienced cardiac tamponade secondary to extramedullary relapse of his multiple myeloma which was limited to the pericardium. In response, his treatment regimen was transitioned to pomalidomide, bortezomib, dexamethasone and cyclophosphamide for three cycles after which he again achieved sCR and ultimately underwent autologous stem cell transplant. Post-transplant consolidation therapy was administered in the form of pomalidomide, bortezomib and dexamethasone, followed by pomalidomide and bortezomib maintenance, which he has continued to receive for 3 years without evidence of disease progression.
在多发性骨髓瘤复发的患者中,超过30%可能患有髓外疾病。髓外多发性骨髓瘤的存在通常与不良细胞遗传学和预后不良相关。最常受累的器官包括肝脏、皮肤、中枢神经系统、胸腔积液、肾脏、淋巴结和胰腺。我们报告一例53岁男性,患有IgA κ型多发性骨髓瘤,具有t(4;14)和1q21扩增的不良细胞遗传学特征,在接受卡非佐米、来那度胺和地塞米松初始治疗后达到了严格完全缓解(sCR)。严格完全缓解的定义是血清中游离轻链比值正常化,骨髓中无克隆细胞,此外还需满足达到完全缓解所需的标准。在计划进行自体干细胞移植之前,该患者因多发性骨髓瘤髓外复发(仅限于心包)而发生心脏压塞。作为应对措施,他的治疗方案转换为泊马度胺、硼替佐米、地塞米松和环磷酰胺,进行三个周期的治疗,之后他再次达到sCR,并最终接受了自体干细胞移植。移植后巩固治疗采用泊马度胺、硼替佐米和地塞米松的形式,随后进行泊马度胺和硼替佐米维持治疗,他已持续接受该治疗3年,无疾病进展迹象。