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首页> 外文期刊>Clinical immunology: The official journal of the Clinical Immunology Society >Immunoglobulin and B-cell disturbances in patients with chronic idiopathic neutropenia
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Immunoglobulin and B-cell disturbances in patients with chronic idiopathic neutropenia

机译：慢性特发性中性粒细胞凋亡患者的免疫球蛋白和B细胞紊乱

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Abstract Chronic idiopathic neutropenia (CIN) is a granulocytic disorder associated with presence of activated, myelosuppressive T-lymphocytes. In the present study we have evaluated constituents of humoral immunity in CIN patients ( n =48) compared to healthy controls ( n =52). CIN patients displayed lower serum IgG levels due to a reduction in IgG1, IgG3, IgG4 but not IgG2, lower IgA and increased IgM levels compared to controls. The proportion of CD19 + cells did not differ between patients and controls; however the proportion of the na?ve IgD + /CD27 ? B-cells was increased and the proportion of class-switched memory IgD ? /CD27 + B-cells was decreased in the patients. The percentage of CD40 + B-cells did not differ between patients and controls and no aberrations in the CD40-meadiated signal transduction pathway or in CD40-gene polymorphisms were identified. These data provide further evidence that immune disturbances are associated with the pathophysiology of CIN and point out for the first time the implication of the B-cell system. Highlights ? Patients with CIN display altered serum immunoglobulin levels. ? Patients with CIN display increased proportion of na?ve IgD + /CD27 ? B-cells. ? Patients with CIN display decreased proportion of class-switched memory IgD ? /CD27 + B-cells. ? The CD40-CD40L expression is normal in CIN. ? The CD40-meadiated signal transduction pathway is normal in CIN.
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