臨床神経学

第51回日本神経学会総会

<Education Program 4>
Crow-Fukase症候群の新規治療展望

桑原 聡

千葉大学大学院医学研究院神経内科学〔〒260―8670 千葉市中央区亥鼻1―8―1〕

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).
Full Text of this Article in Japanese PDF (256K)

(臨床神経, 50:794−796, 2010)
key words:Crow-Fukase症候群,POEMS症候群,自己末梢血幹細胞移植,サリドマイド,抗VEGF抗体

(受付日:2010年5月20日)