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Neutropenia associated with IgA deficiency in a child

Jan Styczyński, Mariusz Wysocki, Małgorzata Kubicka

CaseRepClinPractRev 2003; 4(2):123-127

ID: 429049


Background: Neutropenia develops as a result of lack of effective leukocyte production by the bone marrow and/or excessive destruction of neutrophils in peripheral blood or tissues. In children, it may present as the initial symptom of bone marrow aplasia or acute leukemia; it also constitutes a risk factor for severe, potentially fatal infections. Neutropenia is associated with an increased risk of sepsis caused by both Gram-negative and Gram-positive bacteria. Case Report: A case of an 11-year-old boy with neutropenia accompanied by IgA deficiency is presented. Leukopenia with neutropenia was diagnosed in the course of long-term respiratory mycosis and lamblial infestation of the gastrointestinal tract. Signs of autoimmunization process were also observed. Hematological investigations revealed abnormalities of lymphocyte subpopulation counts, reduced myelopoiesis and normal response in granulocytic system function tests. Persistent IgA deficiency seems to have been the cause of the observed disorders. Production of other antibody classes was normal. Differential diagnostics excluded cyclic, persistent and congenital neutropenias, ‘leukocyte trap’ phenomenon, as well as hypo- and hyperplasias of the bone marrow. Conclusions: IgA deficiency may lead to leukopenia, neutropenia and coincident infections, as well as autoaggressive disorders. Antibiotic therapy and treatment stimulating the immune system favors normalization of the leukocyte counts and control of infections.

Keywords: Leukopenia, Neutropenia, lymphocyte subpopulations, IgA Deficiency, immunostimulation, autoimmunization