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Polyclonal expansion of large granular lymphocytes in common variable immunodeficiency - association with neutropenia.

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 Immunol

journal_title

Clinical and experimental immunology

authors

Holm AM,Tjønnfjord G,Yndestad A,Beiske K,Müller F,Aukrust P,Frøland SS

doi

10.1111/j.1365-2249.2006.03086.x

subject

Has Abstract

pub_date

2006-06-01 00:00:00

pages

418-24

issue

3

eissn

0009-9104

issn

1365-2249

pii

CEI3086

journal_volume

144

pub_type

杂志文章

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