|Aleksandra Szczawińska-Popłonyk, Husam Samara, Anna Bręborowicz,|
Introduction. Hypogammaglobulinemia in infancy and early childhood
is a disorder characterized by complex pathogenesis as well as diverse
clinical manifestations and prognosis. Antibody production defect is
a common result of the activity of various endo- and exogenous factors,
leading to the disturbed peripheral blood lymphocyte homeostasis.
Aim of the study. The main purpose of the study was to elucidate the
pathomechanism of hypogammaglobulinaemia in infants and young
children. The additional purpose of the flow cytometric immunophenotyping
was to define its immunodiagnostic application and to enable
prognosticating the process of development of antibody defects.
Material and methods. Immunophenotyping of peripheral blood B
lymphocyte subsets was performed in twenty-two children with IgG
hypogammaglobulinemia and with or without deficiency of other immunoglobulin
isotypes, aged from 8 to 61 months, suffering from recurrent
respiratory tract infections.
Results. Disorders of differentiation of B lymphocyte precursors at the
stage of bone marrow lymphopoiesis were not identified in the study
group. Increased proportion of transitional B cells was noted in peripheral
blood in 13 of 22 children (59%); the increase was significantly more
frequent in children with defects of immunoglobulin G and A production
(p=0.039); besides, increased relative value of plasmablasts was detected
in 16 children (73%).
Conclusions. Hypogammaglobulinemia in infants and young children is
associated with impaired maturation of peripheral blood B lymphocyte subpopulations.
Flow cytometric immunophenotyping facilitates elimination of
differentiation disorders of B lymphocyte precursors in bone marrow and
identification of peripheral blood B cell maturation defects. Therefore, the
immunophenotyping should be an indispensable element of immunodiagnostic
procedures used to detect antibody production deficiencies.
keywords: niedobór odporności, immunoglobuliny, limfocyty B, dzieci,immunodeficiency, immunoglobulins, B lymphocytes,children
pages: from 97 to 102
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