Restrictive usage of monoclonal immunoglobulin lambda light chain germline in POEMS syndrome.
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.
Abe
Daijiro
D
Division of Hematology, Department of Clinical Cell Biology, Chiba University Graduate School of Medicine, Chiba, Japan.
Nakaseko
Chiaki
C
Takeuchi
Masahiro
M
Tanaka
Hiroaki
H
Ohwada
Chikako
C
Sakaida
Emiko
E
Takeda
Yusuke
Y
Oda
Kayo
K
Ozawa
Shinichi
S
Shimizu
Naomi
N
Masuda
Shinichi
S
Cho
Ryuko
R
Nishimura
Miki
M
Misawa
Sonoko
S
Kuwabara
Satoshi
S
Saito
Yasushi
Y
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
D007135:Immunoglobulin Variable Region; D007146:Immunoglobulin lambda-Chains