Characterization of immunoglobulin λ light chain variable region (IGLV) gene and its relationship with clinical features in patients with POEMS syndrome
- 258 Downloads
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.
KeywordsPOEMS syndrome Immunoglobulin Variable region gene
The authors thank Dr. Wei Su and Ms. Lian-Feng Chen for technical assistance.
This work was supported by a grant from Peking Union Medical College Hospital Youth Foundation (No. 2009117).
- 3.Li J, Zhang W, Jiao L, et al (2011) Combination of melphalan and dexamethasone for patients with newly diagnosed POEMS 2011;117:6445–9Google Scholar
- 6.Aravamudan B, Tong C, Lacy MQ et al (2008) Immunoglobulin variable light chain restriction, cytokine expression and plasma cell-stromal cell interactions in POEMS syndrome patients. Blood 112:2744, abstractGoogle Scholar
- 12.van Dongen JJ, Langerak AW, Brüggemann M et al (2003) Design and standardization of PCR primers and protocols for detection of clonal immunoglobulin and T-cell receptor gene recombinations in suspect lymphoproliferations: report of the BIOMED-2 Concerted Action BMH4-CT 98-3936. Leukemia 17:2257–2317PubMedCrossRefGoogle Scholar