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来那度胺联合小剂量地塞米松治疗自体外周血干细胞移植后复发的POEMS综合征时液体潴留迅速改善

[Rapid improvement of fluid retention with lenalidomide plus low-dose dexamethasone for POEMS syndrome relapsed after autologous peripheral blood stem cell transplantation].

作者信息

Ohashi Kota, Yokoyama Akihiro, Kagoo Toshiya, Hosoda Toru, Yamamoto Ryusuke, Yoneda Mie, Kudo Masahisa, Boku Saigen, Ueno Hironori, Yano Takahiro

机构信息

Division of Hematology, Department of Internal Medicine, National Hospital Organization Tokyo Medical Center.

出版信息

Rinsho Ketsueki. 2013 Jul;54(7):670-4.

Abstract

We report a 53-year-old male with POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome who relapsed after autologous peripheral stem cell transplantation (ASCT), but responded extremely well to lenalidomide (LEN) plus low dose dexamethasone (Ld) therapy. The patient had been diagnosed with POEMS syndrome in November 2006, and underwent ASCT in February 2007. In July 2011, he developed respiratory distress, generalized edema, and massive bilateral pleural effusion. Plasma vascular endothelial growth factor (VEGF) and M protein were increased, strongly indicating a relapse. Ld therapy was remarkably effective for these symptoms, resulting in complete remission with M-protein becoming undetectable by immunofixation. Since completing 11 courses of therapy with an every 4 weeks regimen, he has remained in clinical remission. The patient's activities of daily living have also markedly improved from total physical incapacity to being able to stand with slight assistance. LEN is associated with a lower incidence of peripheral neuropathy than other new drugs. Although LEN has occasionally been given to patients with POEMS syndrome in recent years, there are still few reports on its use for patients with recurrent disease after ASCT. Our successful management of this patient suggests that Ld therapy is not only relatively safe but also a promising option for POEMS syndrome relapsing after ASCT.

摘要

我们报告了一名53岁的男性POEMS(多发性神经病变、器官肿大、内分泌病变、单克隆丙种球蛋白病和皮肤改变)综合征患者,其在自体外周干细胞移植(ASCT)后复发,但对来那度胺(LEN)联合低剂量地塞米松(Ld)治疗反应极佳。该患者于2006年11月被诊断为POEMS综合征,并于2007年2月接受了ASCT。2011年7月,他出现呼吸窘迫、全身水肿和大量双侧胸腔积液。血浆血管内皮生长因子(VEGF)和M蛋白升高,强烈提示复发。Ld治疗对这些症状非常有效,导致完全缓解,免疫固定法检测不到M蛋白。自每4周方案完成11个疗程的治疗以来,他一直处于临床缓解状态。患者的日常生活活动能力也从完全丧失自理能力显著改善为在轻微协助下能够站立。与其他新药相比,LEN引起周围神经病变的发生率较低。尽管近年来偶尔有POEMS综合征患者使用LEN,但关于其用于ASCT后复发患者的报道仍然很少。我们对该患者的成功治疗表明,Ld治疗不仅相对安全,而且是ASCT后复发的POEMS综合征的一个有前景的选择。

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