{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T22:04:31Z","timestamp":1790805871651,"version":"4.1.0"},"reference-count":40,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2018,8,31]],"date-time":"2018-08-31T00:00:00Z","timestamp":1535673600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"Grants-in-Aid for Scientific Research of the Japan Society for the Promotion of Science KAKENHI","award":["15K09494"],"award-info":[{"award-number":["15K09494"]}]},{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science KAKENHI","doi-asserted-by":"crossref","award":["15K09494"],"award-info":[{"award-number":["15K09494"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science KAKENHI","doi-asserted-by":"crossref","award":["18K08338"],"award-info":[{"award-number":["18K08338"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"crossref"}]},{"name":"2015 Japanese Society of Myeloma Research Award (E.S.)"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[2018,9]]},"abstract":"<jats:p>Polyneuropathy, organomegaly, endocrinopathy, M\u2010protein, and skin changes (POEMS) syndrome is a rare plasma cell dyscrasia characterized by polyneuropathy, organomegaly, endocrinopathy, extravascular fluid overload, M protein, and a myriad of skin changes. The pathogenesis is poorly understood, but monoclonal plasma cells are \u03bb\u2010restricted and these immunoglobulin \u03bb light chain variable (IGLV) region genes are derived from only two germlines, either <jats:italic>IGLV1\u201044<\/jats:italic> or <jats:italic>1\u201040<\/jats:italic>. Here we analyzed the clonal <jats:italic>IGLV<\/jats:italic> gene rearrangements of genomic DNA samples of bone marrow mononuclear cells using next\u2010generation sequencing (NGS) to understand the clonal composition of <jats:italic>IGLV<\/jats:italic> genes in patients with POEMS syndrome (n\u2009=\u200930). The dominant <jats:italic>IGLV<\/jats:italic> gene rearrangement of POEMS syndrome\u2010specific germline sequences were significantly increased in 11 POEMS patients (36.7%; <jats:italic>IGLV1\u201044<\/jats:italic>: n\u2009=\u20099, <jats:italic>IGLV1\u201040<\/jats:italic>: n\u2009=\u20092). In some cases, <jats:italic>IGLV<\/jats:italic> gene rearrangement clone was not detected as significant increase but was detected using cDNA samples by heteroduplex (HD) analysis and Sanger sequencing, suggesting that the quite small number of monoclonal plasma cells may produce large quantity of mRNA of monoclonal proteins. However, significant increase of dominant clone sizes was not directly linked to the initial disease status. On the other hand, in cases with significantly increased dominant clones, they decreased and increased accompanying with disease remission and relapse. These data demonstrate that monoclonal plasma cells are related to the pathogenesis of POEMS syndrome.<\/jats:p>","DOI":"10.1002\/ajh.25213","type":"journal-article","created":{"date-parts":[[2018,7,17]],"date-time":"2018-07-17T11:16:43Z","timestamp":1531826203000},"page":"1161-1168","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":22,"title":["Clonal immunoglobulin \u03bb light\u2010chain gene rearrangements detected by next generation sequencing in POEMS syndrome"],"prefix":"10.1002","volume":"93","author":[{"given":"Chika","family":"Kawajiri\u2010Manako","sequence":"first","affiliation":[{"name":"Department of Hematology Chiba University Hospital  Chiba Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Naoya","family":"Mimura","sequence":"additional","affiliation":[{"name":"Department of Hematology Chiba University Hospital  Chiba Japan"},{"name":"Department of Transfusion Medicine and Cell Therapy Chiba 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