Department of Hematology and Oncology, University Hospital Wuerzburg, Oberdürrbacher Str. 6, 97080 Wuerzburg, Germany.
Ann Hematol. 2012 Jul;91(7):1031-7. doi: 10.1007/s00277-012-1414-5.
Extramedullary (e) relapse in multiple myeloma(MM) has an adverse prognosis, but knowledge concerning biological features and preferred treatment is scarce. We screened the myeloma registry of our institution for eMM relapses and identified 24 cases among 357 patients (pts).Only 8% of eMM relapses occurred after initial therapy, but 54% occurred after third-line or subsequent therapy. Baseline molecular cytogenetics revealed high-risk features in 10 of 19 evaluable patients. Most frequently, eMM presented as soft tissue (67%) and organ involvement (25%) or malignant effusion (12.5%). Incidence of leptomeningeal/CNS involvement was 21%. At eMM relapse, bone marrow infiltration was absent in 46% and low in 21%. Ten eMM biopsies were available showing increased proliferation, i.e., Ki-67 of 67%(range, 30–90%) of all cancer cells. Pts received radiation therapy, dose-intense chemotherapy, novel agents, and allogeneic SCT resulting in an overall response rate of 54%. Median progression-free survival was 2 (95% CI 0.08–3.92) and median overall survival 7 months (95% CI 3.56–10.43), respectively,with only three patients being alive at 12 months from diagnosis. EMM relapse may present at any anatomical site with frequent CNS involvement. Biological features include increased proliferation and low rate of marrow involvement.Prognosis remains poor despite intensive treatment.
髓外（e）多发性骨髓瘤（MM）复发预后不良，但对其生物学特征和首选治疗方法知之甚少。我们对本机构的骨髓瘤登记处进行了筛选，在 357 例患者中发现了 24 例 eMM 复发患者。仅有 8%的 eMM 复发发生在初始治疗后，但 54%发生在三线或后续治疗后。基线分子细胞遗传学显示，19 例可评估患者中有 10 例存在高危特征。最常见的是软组织（67%）和器官受累（25%）或恶性积液（12.5%）。软脑膜/中枢神经系统受累的发生率为 21%。在 eMM 复发时，骨髓浸润缺失的占 46%，低水平的占 21%。有 10 例 eMM 活检可查，显示增殖增加，即所有癌细胞的 Ki-67 为 67%（范围为 30-90%）。患者接受了放疗、剂量密集化疗、新型药物和异基因 SCT，总缓解率为 54%。无进展生存期的中位数为 2 个月（95%CI 0.08-3.92），总生存期的中位数为 7 个月（95%CI 3.56-10.43），诊断后 12 个月时仅有 3 例患者存活。eMM 复发可发生在任何解剖部位，且常有中枢神经系统受累。生物学特征包括增殖增加和骨髓受累率低。尽管进行了强化治疗，但预后仍较差。
