Immunoglobulin and B-cell disturbances in patients with chronic idiopathic neutropenia

Publication date: Available online 18 July 2017
Source:Clinical Immunology
Author(s): Irene Mavroudi, Aristides G. Eliopoulos, Charalampos Pontikoglou, Katerina Pyrovolaki, Athina Damianaki, Helen Koutala, Maria I. Zervou, Maria Ximeri, Semeli Mastrodemou, Peggy Kanellou, George N. Goulielmos, Helen A. Papadaki
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 naïve 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.



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