title: Restrictive usage of monoclonal immunoglobulin lambda light chain germline in POEMS syndrome.
abstract: 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.
authors.affiliation: Division of Hematology, Department of Clinical Cell Biology, Chiba University Graduate School of Medicine, Chiba, Japan.
authors.forename: Daijiro
authors.initials: D
authors.lastname: Abe
authors.forename: Chiaki
authors.initials: C
authors.lastname: Nakaseko
authors.forename: Masahiro
authors.initials: M
authors.lastname: Takeuchi
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Tanaka
authors.forename: Chikako
authors.initials: C
authors.lastname: Ohwada
authors.forename: Emiko
authors.initials: E
authors.lastname: Sakaida
authors.forename: Yusuke
authors.initials: Y
authors.lastname: Takeda
authors.forename: Kayo
authors.initials: K
authors.lastname: Oda
authors.forename: Shinichi
authors.initials: S
authors.lastname: Ozawa
authors.forename: Naomi
authors.initials: N
authors.lastname: Shimizu
authors.forename: Shinichi
authors.initials: S
authors.lastname: Masuda
authors.forename: Ryuko
authors.initials: R
authors.lastname: Cho
authors.forename: Miki
authors.initials: M
authors.lastname: Nishimura
authors.forename: Sonoko
authors.initials: S
authors.lastname: Misawa
authors.forename: Satoshi
authors.initials: S
authors.lastname: Kuwabara
authors.forename: Yasushi
authors.initials: Y
authors.lastname: Saito
mesh_terms: D000328:Adult; D000368:Aged; D001483:Base Sequence; D005260:Female; D005854:Germ Cells; D006801:Humans; D007135:Immunoglobulin Variable Region / Q000235:genetics; D007146:Immunoglobulin lambda-Chains / Q000235:genetics*; D008297:Male; D008875:Middle Aged; D016878:POEMS Syndrome / Q000209:etiology*; D017385:Sequence Homology
chemical_list: D007135:Immunoglobulin Variable Region; D007146:Immunoglobulin lambda-Chains