Kuwabara Satoshi
Department of Neurology, Graduate School of Medicine, Chiba University.
Rinsho Shinkeigaku. 2010 Nov;50(11):794-6. doi: 10.5692/clinicalneurol.50.794.
Crow-Fukase syndrome, also called POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome, is a rare cause of demyelinating and axonal mixed neuropathy with multiorgan involvement. The pathogenesis of Crow-Fukase syndrome is not well understood, but overproduction of vascular endothelial growth factor (VEGF), probably mediated by monoclonal proliferation of plasma cells, is likely to be responsible for most of the characteristic symptoms. There is no established treatment regimen. In appropriate candidates, high-dose chemotherapies with autologous peripheral blood stem cell transplantation is highly recommended, because this treatment could result in obvious improvement in neuropathy as well as other symptoms, with a significant decrease in serum VEGF levels. Indication of this treatment has not yet been established, and long-term prognosis is unclear at present. Thalidomide should be considered for patients who are not indicated for transplantation therapy. Treatments that should be considered as future therapy include lenalidomide, bortezomib, and anti-VEGF monoclonal antibody (bevacizumab).
克劳-富卡塞综合征，也称为POEMS（多发性神经病、器官肿大、内分泌病、M蛋白和皮肤改变）综合征，是一种罕见的脱髓鞘和轴索性混合性神经病病因，可累及多个器官。克劳-富卡塞综合征的发病机制尚不完全清楚，但血管内皮生长因子（VEGF）过度产生，可能由浆细胞单克隆增殖介导，很可能是导致大多数特征性症状的原因。目前尚无既定的治疗方案。对于合适的患者，强烈推荐采用高剂量化疗联合自体外周血干细胞移植，因为这种治疗可使神经病及其他症状明显改善，血清VEGF水平显著降低。这种治疗的适应证尚未确定，目前长期预后尚不清楚。对于不适合移植治疗的患者，应考虑使用沙利度胺。应作为未来治疗手段考虑的治疗方法包括来那度胺、硼替佐米和抗VEGF单克隆抗体（贝伐单抗）。
