Background and Pathophysiology of Autoimmune Neutropenia
Autoimmune neutropenia is estimated to occur in at least 1 in 100,000 children. The majority of patients are affected during the first 2 years of life, with resolution by age 5. Primary autoimmune neutropenia of infancy may present at the time of an acute infection or incidentally when a complete blood count is obtained for another reason. The neutropenia is a result of peripheral destruction due to autoantibody production against antigens on the neutrophil surface, most commonly human neutrophil antigen 1 on the neutrophil immunoglobulin receptor FcγRIIIb. Antibodies often remit within 18–24 months from the time of diagnosis. Most children experience a benign course with limited increased risk of infections. In contrast, secondary autoimmune neutropenia may present at any age with a more variable clinical course. Patients with the latter may have pan-FcγRIIIb autoantibodies and should undergo investigation for other autoimmune or primary immune system disorders.
KeywordsAutoimmune neutropenia Chronic benign neutropenia of childhood
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