Department of Internal Medicine and Clinical Immunology, Yokohama City University Graduate School of Medicine, 3-9 Fukuura, Kanazawa, Yokohama, 236-0004, Japan,
Int J Hematol. 2013 Dec;98(6):723-8. doi: 10.1007/s12185-013-1456-z. Epub 2013 Oct 29.
POEMS syndrome is a monoclonal plasma cell disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. High-dose therapy (HDT) and autologous stem cell transplantation (ASCT) are an effective therapy, but optimal treatment options are still under debate. Bortezomib is an important agent for the treatment of patients with multiple myeloma and has recently been reported as efficacious in the treatment of patients with POEMS syndrome. We present a case of POEMS syndrome in a 33-year-old woman, who was successfully treated with BorDex (bortezomib and dexamethasone) combined with radiotherapy, and followed by ASCT. She was diagnosed with POEMS syndrome with a localized plasmacytoma of bone 5 months after her initial symptoms of heart failure. Her Eastern Cooperative Oncology Group (ECOG) performance status was 4. She was first administered BorDex therapy, which was subsequently combined with radiotherapy. Her general condition including heart failure dramatically improved after four cycles of BorDex therapy and radiation, resulting in partial response. After chemoradiotherapy, HDT and ASCT were performed. After treatment, she was able to walk unassisted and her plasma endothelial growth factor (VEGF) level decreased. She did not experience neurotoxicity induced by bortezomib. Bortezomib was well tolerated and we suggest that BorDex therapy followed by HDT and ASCT may be an effective therapy for POEMS syndrome.
POEMS 综合征是一种单克隆浆细胞疾病，其特征为多发性神经病、脏器肿大、内分泌病、单克隆丙种球蛋白病和皮肤改变。大剂量化疗（HDT）和自体干细胞移植（ASCT）是一种有效的治疗方法，但最佳治疗方案仍存在争议。硼替佐米是治疗多发性骨髓瘤患者的重要药物，最近有报道称其对 POEMS 综合征患者有效。我们报告了 1 例 33 岁女性 POEMS 综合征病例，她成功接受了 BorDex（硼替佐米和地塞米松）联合放疗，随后进行了 ASCT。她在最初出现心力衰竭症状 5 个月后被诊断为 POEMS 综合征合并骨局部浆细胞瘤。她的东部肿瘤协作组（ECOG）体能状态评分为 4 分。她首先接受了 BorDex 治疗，随后联合放疗。在 BorDex 治疗和放疗的四个周期后，她的一般状况（包括心力衰竭）明显改善，达到部分缓解。化疗和放疗后，进行了 HDT 和 ASCT。治疗后，她能够独立行走，血浆内皮生长因子（VEGF）水平降低。她没有经历硼替佐米引起的神经毒性。硼替佐米耐受性良好，我们建议 BorDex 治疗后进行 HDT 和 ASCT 可能是 POEMS 综合征的有效治疗方法。
