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Annals of Hematology

, Volume 91, Issue 8, pp 1251–1255 | Cite as

Characterization of immunoglobulin λ light chain variable region (IGLV) gene and its relationship with clinical features in patients with POEMS syndrome

  • Jian Li
  • Zhen Huang
  • Ming-hui Duan
  • Wei Zhang
  • Miao Chen
  • Xin-xin Cao
  • Hong-zhi Guan
  • Zhuang Tian
  • Dao-bin ZhouEmail author
Original Article

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.

Keywords

POEMS syndrome Immunoglobulin Variable region gene 

Notes

Acknowledgments

The authors thank Dr. Wei Su and Ms. Lian-Feng Chen for technical assistance.

Funding

This work was supported by a grant from Peking Union Medical College Hospital Youth Foundation (No. 2009117).

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Copyright information

© Springer-Verlag 2012

Authors and Affiliations

  • Jian Li
    • 1
  • Zhen Huang
    • 1
  • Ming-hui Duan
    • 1
  • Wei Zhang
    • 1
  • Miao Chen
    • 1
  • Xin-xin Cao
    • 1
  • Hong-zhi Guan
    • 2
  • Zhuang Tian
    • 3
  • Dao-bin Zhou
    • 1
    Email author
  1. 1.Department of Hematology, Peking Union Medical College HospitalChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijingPeople’s Republic of China
  2. 2.Department of Neurology, Peking Union Medical College HospitalChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijingPeople’s Republic of China
  3. 3.Department of Cardiology, Peking Union Medical College HospitalChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijingPeople’s Republic of China

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