Familial monoclonal gammopathy: hyper‐responsive B cells in unaffected family members
Abstract Background: In Iceland, eight families have been identified with multiple cases of monoclonal gammopathies (MG) and other lymphoproliferative diseases. In one of these families with several cases of monoclonal gammopathy of undetermined significance (MGUS) and Waldenströms macroglobulinemia...
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crwiley:10.1111/j.1600-0609.2011.01593.x 2023-12-03T10:24:59+01:00 Familial monoclonal gammopathy: hyper‐responsive B cells in unaffected family members Steingrímsdóttir, Hlíf Einarsdóttir, Helga K. Haraldsdóttir, Vilhelmína Ögmundsdóttir, Helga M. 2011 http://dx.doi.org/10.1111/j.1600-0609.2011.01593.x https://api.wiley.com/onlinelibrary/tdm/v1/articles/10.1111%2Fj.1600-0609.2011.01593.x https://onlinelibrary.wiley.com/doi/pdf/10.1111/j.1600-0609.2011.01593.x en eng Wiley http://onlinelibrary.wiley.com/termsAndConditions#vor European Journal of Haematology volume 86, issue 5, page 396-404 ISSN 0902-4441 1600-0609 Hematology General Medicine journal-article 2011 crwiley https://doi.org/10.1111/j.1600-0609.2011.01593.x 2023-11-09T13:55:32Z Abstract Background: In Iceland, eight families have been identified with multiple cases of monoclonal gammopathies (MG) and other lymphoproliferative diseases. In one of these families with several cases of monoclonal gammopathy of undetermined significance (MGUS) and Waldenströms macroglobulinemia, in vitro stimulation with poke‐weed mitogen revealed hyper‐responsive B cells showing increased immunoglobulin production in one‐third of disease‐free family members. Design and methods: In this study, the families were further traced and the list of names produced was compared with The Icelandic Cancer Registry (ICR) to find all recent cases of lymphoproliferative diseases. First‐degree relatives and descendants older than 20 yrs of age ( n = 350) were selected for screening for paraprotein. Selected family members were tested for B‐cell hyper‐responsiveness and the lymphocyte phenotype was analysed by flow cytometry. Results: Comparison of the total list of 4370 family members with the ICR revealed 22 new cases and screening for serum paraprotein identified nine new cases of MG, eight being first‐degree relatives of known probands. Sixty cases of lymphoproliferative diseases are currently known within the eight families, five of them containing both IgG/A and IgM disorders. Twelve hyper‐responders (HR) were identified in four families, eight from one family, of whom four were known already. Stimulated B cells from HR had a significantly higher proportion of CD27 + memory/plasma cells than controls. Conclusion: Identification of new affected family members by screening confirms a hereditary predisposition to B‐cell proliferative diseases. Contrary to most studies, IgG/A and IgM disorders occurred together in five families. In four families, enhanced B‐cell responsiveness was found in healthy subjects clustered around cases. Article in Journal/Newspaper Iceland Wiley Online Library (via Crossref) European Journal of Haematology 86 5 396 404 |
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English |
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Hematology General Medicine |
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Hematology General Medicine Steingrímsdóttir, Hlíf Einarsdóttir, Helga K. Haraldsdóttir, Vilhelmína Ögmundsdóttir, Helga M. Familial monoclonal gammopathy: hyper‐responsive B cells in unaffected family members |
topic_facet |
Hematology General Medicine |
description |
Abstract Background: In Iceland, eight families have been identified with multiple cases of monoclonal gammopathies (MG) and other lymphoproliferative diseases. In one of these families with several cases of monoclonal gammopathy of undetermined significance (MGUS) and Waldenströms macroglobulinemia, in vitro stimulation with poke‐weed mitogen revealed hyper‐responsive B cells showing increased immunoglobulin production in one‐third of disease‐free family members. Design and methods: In this study, the families were further traced and the list of names produced was compared with The Icelandic Cancer Registry (ICR) to find all recent cases of lymphoproliferative diseases. First‐degree relatives and descendants older than 20 yrs of age ( n = 350) were selected for screening for paraprotein. Selected family members were tested for B‐cell hyper‐responsiveness and the lymphocyte phenotype was analysed by flow cytometry. Results: Comparison of the total list of 4370 family members with the ICR revealed 22 new cases and screening for serum paraprotein identified nine new cases of MG, eight being first‐degree relatives of known probands. Sixty cases of lymphoproliferative diseases are currently known within the eight families, five of them containing both IgG/A and IgM disorders. Twelve hyper‐responders (HR) were identified in four families, eight from one family, of whom four were known already. Stimulated B cells from HR had a significantly higher proportion of CD27 + memory/plasma cells than controls. Conclusion: Identification of new affected family members by screening confirms a hereditary predisposition to B‐cell proliferative diseases. Contrary to most studies, IgG/A and IgM disorders occurred together in five families. In four families, enhanced B‐cell responsiveness was found in healthy subjects clustered around cases. |
format |
Article in Journal/Newspaper |
author |
Steingrímsdóttir, Hlíf Einarsdóttir, Helga K. Haraldsdóttir, Vilhelmína Ögmundsdóttir, Helga M. |
author_facet |
Steingrímsdóttir, Hlíf Einarsdóttir, Helga K. Haraldsdóttir, Vilhelmína Ögmundsdóttir, Helga M. |
author_sort |
Steingrímsdóttir, Hlíf |
title |
Familial monoclonal gammopathy: hyper‐responsive B cells in unaffected family members |
title_short |
Familial monoclonal gammopathy: hyper‐responsive B cells in unaffected family members |
title_full |
Familial monoclonal gammopathy: hyper‐responsive B cells in unaffected family members |
title_fullStr |
Familial monoclonal gammopathy: hyper‐responsive B cells in unaffected family members |
title_full_unstemmed |
Familial monoclonal gammopathy: hyper‐responsive B cells in unaffected family members |
title_sort |
familial monoclonal gammopathy: hyper‐responsive b cells in unaffected family members |
publisher |
Wiley |
publishDate |
2011 |
url |
http://dx.doi.org/10.1111/j.1600-0609.2011.01593.x https://api.wiley.com/onlinelibrary/tdm/v1/articles/10.1111%2Fj.1600-0609.2011.01593.x https://onlinelibrary.wiley.com/doi/pdf/10.1111/j.1600-0609.2011.01593.x |
genre |
Iceland |
genre_facet |
Iceland |
op_source |
European Journal of Haematology volume 86, issue 5, page 396-404 ISSN 0902-4441 1600-0609 |
op_rights |
http://onlinelibrary.wiley.com/termsAndConditions#vor |
op_doi |
https://doi.org/10.1111/j.1600-0609.2011.01593.x |
container_title |
European Journal of Haematology |
container_volume |
86 |
container_issue |
5 |
container_start_page |
396 |
op_container_end_page |
404 |
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1784273608832450560 |