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Clinical immunology : the official journal of the Clinical Immunology Society v.183, 2017년, pp.75 - 81   SCI SCIE SCOPUS
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Immunoglobulin and B-cell disturbances in patients with chronic idiopathic neutropenia

Mavroudi, Irene (Department of Hematology, School of Medicine, University of Crete, Greece ) ; Eliopoulos, Aristides G. (Molecular and Cellular Biology Laboratory, School of Medicine, University of Crete, Heraklion, Greece ) ; Pontikoglou, Charalampos (Department of Hematology, School of Medicine, University of Crete, Greece ) ; Pyrovolaki, Katerina (Department of Hematology, School of Medicine, University of Crete, Greece ) ; Damianaki, Athina (Department of Hematology, School of Medicine, University of Crete, Greece ) ; Koutala, Helen (Department of Hematology, School of Medicine, University of Crete, Greece ) ; Zervou, Maria I. (Department of Internal Medicine, School of Medicine, University of Crete, Greece ) ; Ximeri, Maria (Department of Hematology, School of Medicine, University of Crete, Greece ) ; Mastrodemou, Semeli (Department of Hematology, School of Medicine, University of Crete, Greece ) ; Kanellou, Peggy (Department of Hematology, School of Medicine, University of Crete, Greece ) ; Goulielmos, George N. (Department of Internal Medicine, School of Medicine, University of Crete, Greece ) ; Papadaki, Helen A. (Department of Hematology, School of Medicine, University of Crete, Greece ) ;
  • 초록  

    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 naIve 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 naIve 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.


  • 주제어

    Chronic idiopathic neutropenia .   Immunoglobulin disturbances .   IgD/CD27 B-cells .   CD40 .   CD40 ligand.  

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