Submitted December 5, 2007
Accepted April 19, 2008
Restrictive usage of monoclonal immunoglobulin 
light chain germline in POEMS syndrome
Daijiro Abe, Chiaki Nakaseko*, Masahiro Takeuchi, Hiroaki Tanaka, Chikako Ohwada, Emiko Sakaida, Yusuke Takeda, Kayo Oda, Shinichi Ozawa, Naomi Shimizu, Shinichi Masuda, Ryuko Cho, Miki Nishimura, Sonoko Misawa, Satoshi Kuwabara, and Yasushi Saito
Division of Hematology, Department of Clinical Cell Biology, Chiba University Graduate School of Medicine, Chiba, Japan
Department of Neurology, Chiba University Graduate School of Medicine, Chiba, Japan
* Corresponding author; email: chiaki-nakaseko{at}faculty.chiba-u.jp.
POEMS syndrome is a rare plasma cell disorder characterized by peripheral neuropathy, monoclonal gammopathy, and high levels of serum vascular endothelial growth factor (VEGF), the pathogenesis of which remains unclear. A unique feature of this syndrome is that the proliferating monoclonal plasma cells are essentially 
-restricted. Here, we determined complete nucleotide sequences of monoclonal immunoglobulin light chain (IGL) variable regions in 11 patients with POEMS syndrome. The V-region of the Ig gene of all 11 patients was restricted to the V1 subfamily. Searching for homologies with IGL germlines revealed that two germlines, IGLV1-44*01 (9/11) and IGLV1-40*01 (2/10) were identified, with an average homology of 91.1%. The IGLJ3*02 gene was used in 11/11 rearrangements with an average homology of 92.2%. These data suggest that the highly restricted use of IGL V1 germlines plays an important role in the pathogenesis of POEMS syndrome.
