Imai Noboru, Kitamura Eiji, Tachibana Takayoshi, Konishi Takashi, Suzuki Youji, Serizawa Masahiro, Okabe Takashi, Fujita Hiroyuki
The Department of Neurology, Shizuoka Red Cross Hospital.
Intern Med. 2007;46(3):135-8. doi: 10.2169/internalmedicine.46.1713. Epub 2007 Feb 1.
POEMS syndrome (POEMS) is a rare multi-system disease which is typically associated with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes. We report herein a case of POEMS with progressive polyneuropathy resistant to high-dose intravenous immunoglobulin therapy who experienced improvement in polyneuropathy after the administration of high-dose chemotherapy followed by autologous peripheral blood stem cell transplantation. Improvements in polyneuropathy remain despite recognition of monoclonal gammopathy by immunofixation as of 1 year after treatment. We demonstrated that autologous peripheral blood stem cell transplantation can improve clinical symptoms and signs due to polyneuropathy in POEMS even without achieving complete remission.
POEMS综合征（POEMS）是一种罕见的多系统疾病，通常与多发性神经病、脏器肿大、内分泌病、单克隆丙种球蛋白病和皮肤改变相关。我们在此报告一例POEMS综合征患者，其患有对大剂量静脉注射免疫球蛋白治疗耐药的进行性多发性神经病，在接受大剂量化疗后进行自体外周血干细胞移植，多发性神经病症状得到改善。治疗后1年通过免疫固定法仍可检测到单克隆丙种球蛋白病，但多发性神经病症状持续改善。我们证明，自体外周血干细胞移植即使未实现完全缓解，也可改善POEMS综合征中由多发性神经病引起的临床症状和体征。
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