Abstract:
:Common variable immunodeficiency (CVID) is the most frequent symptomatic primary immunodeficiency disease, characterized by low levels of circulating immunoglobulins and recurrent bacterial infections, particularly of the respiratory tract. T cell dysfunction is often present, and lymphoproliferative and autoimmune disorders as well as haematological cytopenias are frequently observed. In this study, we report a polyclonal expansion of large granular lymphocytes (LGL) in a substantial proportion of CVID patients, associated with splenomegaly, increased numbers of CD8(+) T cells, inverted CD4 : CD8 T cell ratios and neutropenia. CVID patients who had both increased numbers of LGL and granulocytopenia had elevated levels of soluble Fas ligand (sFasL). Our observations indicate that CVID may be added to the list of inflammatory diseases associated with increased numbers of LGL. Furthermore, our findings suggest common pathogenic mechanisms of granulocytopenia in CVID and lymphoproliferative disease of granular lymphocytes.
journal_name
Clin Exp Immunoljournal_title
Clinical and experimental immunologyauthors
Holm AM,Tjønnfjord G,Yndestad A,Beiske K,Müller F,Aukrust P,Frøland SSdoi
10.1111/j.1365-2249.2006.03086.xsubject
Has Abstractpub_date
2006-06-01 00:00:00pages
418-24issue
3eissn
0009-9104issn
1365-2249pii
CEI3086journal_volume
144pub_type
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journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:
更新日期:1977-05-01 00:00:00
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journal_title:Clinical and experimental immunology
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更新日期:1981-05-01 00:00:00
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journal_title:Clinical and experimental immunology
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journal_title:Clinical and experimental immunology
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doi:
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journal_title:Clinical and experimental immunology
pub_type: 杂志文章
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更新日期:1987-01-01 00:00:00
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journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:
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journal_title:Clinical and experimental immunology
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更新日期:1982-09-01 00:00:00
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pub_type: 杂志文章
doi:
更新日期:1982-02-01 00:00:00
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journal_title:Clinical and experimental immunology
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