title: Characterization of immunoglobulin λ light chain variable region (IGLV) gene and its relationship with clinical features in patients with POEMS syndrome.
abstract: The M protein of POEMS syndrome is essentially λ light chain restricted. Several studies have demonstrated the restrict usage of immunoglobulin λ light chain variable region (IGLV) genes in patients with POEMS syndrome. However, these studies only included a limited number of cases, and it is not clear whether the clinical features are influenced by the IGLV gene in POEMS syndrome. Here we demonstrated that the clonal IGLV genes were strictly derived from IGLV 1-40 (11 patients, 36.7 %) and IGLV 1-44 (19, 63.3 %) gene in 30 patients with POEMS syndrome. We further evaluated the relationship between clinical features and IGLV genes. Our study showed that patients with IGLV 1-44 were older than those with IGLV 1-40, and patients with IGLV 1-40 had more severe neuropathy, hypertrichosis, and papilledema. It was suggested that the IGLV gene influenced clinical characteristics in POEMS syndrome.
authors.affiliation: Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, People's Republic of China.
authors.forename: Jian
authors.initials: J
authors.lastname: Li
authors.forename: Zhen
authors.initials: Z
authors.lastname: Huang
authors.forename: Ming-hui
authors.initials: MH
authors.lastname: Duan
authors.forename: Wei
authors.initials: W
authors.lastname: Zhang
authors.forename: Miao
authors.initials: M
authors.lastname: Chen
authors.forename: Xin-xin
authors.initials: XX
authors.lastname: Cao
authors.forename: Hong-zhi
authors.initials: HZ
authors.lastname: Guan
authors.forename: Zhuang
authors.initials: Z
authors.lastname: Tian
authors.forename: Dao-bin
authors.initials: DB
authors.lastname: Zhou
mesh_terms: D000328:Adult; D000368:Aged; D015331:Cohort Studies; D004252:DNA Mutational Analysis / Q000379:methods; D003937:Diagnosis, Differential; D005260:Female; D015321:Gene Rearrangement / Q000235:genetics / Q000276:immunology; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000032:analysis / Q000235:genetics; D007135:Immunoglobulin Variable Region / Q000032:analysis / Q000235:genetics*; D007146:Immunoglobulin lambda-Chains / Q000032:analysis / Q000235:genetics*; D008297:Male; D008875:Middle Aged; D016878:POEMS Syndrome / Q000175:diagnosis* / Q000235:genetics* / Q000276:immunology; D011379:Prognosis; D055815:Young Adult
chemical_list: D007147:Immunoglobulin Light Chains; D007135:Immunoglobulin Variable Region; D007146:Immunoglobulin lambda-Chains