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[自体外周血干细胞移植成功治疗后出现短暂免疫球蛋白同种型转换的POEMS综合征]

[POEMS syndrome presenting with transient immunoglobulin isotype switching after successful treatment with autologous peripheral blood stem cell transplantation].

作者信息

Nakamura Yuichi, Sato Yasutaka, Ito Yoshihiro, Maeda Tomoya, Takahashi Naoki, Kawai Nobutaka, Jinnai Itsuro, Bessho Masami, Kinoshita Shunsuke, Yamamoto Toshimasa

机构信息

Division of Hematology, Saitama Medical University.

出版信息

Rinsho Ketsueki. 2007 Aug;48(8):642-6.

Abstract

A 60-year-old man was admitted with muscle weakness and numbness in the extremities. Based on the existence of monoclonal gammopathy of the IgG-lamda type, a slight increase of plasma cells in the bone marrow, and an elevated level of serum vascular endothelial growth factor (VEGF), the diagnosis of POEMS syndrome was made. After peripheral blood stem cell collection by etoposide and G-CSF, the patient received high dose melphalan (200 mg/m2) therapy supported by autologous peripheral blood stem cell transplantation (autoPBSCT). After high-dose chemotherapy with autoPBSCT, the serum VEGF level normalized and the monoclonal IgG-lamda, disappeared. The patient gradually recovered from a bedridden state and at the time of writing has no impairment in his activities of daily life. After the autoPBSCT, monoclonal IgG-kappa, protein was detected transiently in serum. The new monoclonal immunoglobulin was considered to be due to normal immune reconstitution after myeloablation rather than alteration of the abnormal plasma cell clone, similarly as oligoclonal immunoglobulins occur in multiple myeloma after autoPBSCT. AutoPBSCT with high-dose chemotherapy should be considered among the treatments of choice for POEMS syndrome.

摘要

一名60岁男性因肢体肌肉无力和麻木入院。基于存在IgG-λ型单克隆丙种球蛋白病、骨髓中浆细胞轻度增多以及血清血管内皮生长因子(VEGF)水平升高,诊断为POEMS综合征。在使用依托泊苷和粒细胞集落刺激因子(G-CSF)采集外周血干细胞后,患者接受了大剂量美法仑(200 mg/m²)治疗,并辅以自体外周血干细胞移植(autoPBSCT)。经过大剂量化疗及autoPBSCT后,血清VEGF水平恢复正常,单克隆IgG-λ消失。患者逐渐从卧床状态恢复,在撰写本文时日常生活活动无受损情况。autoPBSCT后,血清中短暂检测到单克隆IgG-κ蛋白。新的单克隆免疫球蛋白被认为是由于清髓后正常免疫重建所致,而非异常浆细胞克隆的改变,这与autoPBSCT后多发性骨髓瘤中出现寡克隆免疫球蛋白的情况类似。对于POEMS综合征的治疗,应考虑将大剂量化疗联合autoPBSCT作为首选治疗方法之一。

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