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 Blood, 1 August 2008, Vol. 112, No. 3, pp. 836-839.
Prepublished online as a Blood First Edition Paper on May 22, 2008; DOI 10.1182/blood-2007-12-126979.

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NEOPLASIA

Brief Report

Restrictive usage of monoclonal immunoglobulin {lambda} light chain germline in POEMS syndrome

Daijiro Abe1, Chiaki Nakaseko1, Masahiro Takeuchi1, Hiroaki Tanaka1, Chikako Ohwada1, Emiko Sakaida1, Yusuke Takeda1, Kayo Oda1, Shinichi Ozawa1, Naomi Shimizu1, Shinichi Masuda1, Ryuko Cho1, Miki Nishimura1, Sonoko Misawa2, Satoshi Kuwabara2, and Yasushi Saito1

1 Division of Hematology, Department of Clinical Cell Biology, and 2 Department of Neurology, Chiba University Graduate School of Medicine, Chiba, Japan

POEMS syndrome is a rare plasma cell disorder characterized by peripheral neuropathy, monoclonal gammopathy, and high levels of serum vascular endothelial growth factor, the pathogenesis of which remains unclear. A unique feature of this syndrome is that the proliferating monoclonal plasma cells are essentially {lambda}-restricted. Here we determined complete nucleotide sequences of monoclonal immunoglobulin {lambda} light chain (IGL) variable regions in 11 patients with POEMS syndrome. The V-region of the Ig{lambda} gene of all 11 patients was restricted to the V{lambda}1 subfamily. Searching for homologies with IGL germlines revealed that 2 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 of 11 re-arrangements with an average homology of 92.2%. These data suggest that the highly restricted use of IGL V{lambda}1 germlines plays an important role in the pathogenesis of POEMS syndrome.


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