RESUMO
Primary autoimmune neutropenia (AIN) is characterised by severe neutropenia and the presence of granulocyte reactive autoantibodies. The pathogenesis of the disease remains unknown and the disease is believed to be underdiagnosed. AIN occurs predominantly at the age of 6-24 months and despite severe neutropenia the symptomatology covers mainly benign infections. Serious bacterial infections might occur and some patients thus may benefit from treatment with prophylactic antibiotics or granulocyte growth factor. Spontaneous remission usually occurs within 30 months from the time of diagnosis.
Assuntos
Doenças Autoimunes , Neutropenia , Doenças Autoimunes/diagnóstico , Doenças Autoimunes/tratamento farmacológico , Doenças Autoimunes/etiologia , Criança , Pré-Escolar , Diagnóstico Diferencial , Humanos , Lactente , Neutropenia/diagnóstico , Neutropenia/tratamento farmacológico , Neutropenia/etiologia , Neutropenia/imunologiaRESUMO
Case reports of three children with autoimmune neutropenia are presented. The children had a number of infections, which required antibiotic treatment, mostly respiratory tract infections, but one child had a septic coxitis. During a follow-up period of 1-3 years, only one patient showed complete remission after 15 months of neutropenia. One child was treated with granulocyte colony-stimulating factor without effect, whereas two children received prophylactic antibiotics with clinical benefit. In view of the frequent infections and benefit of prophylactic measures, children with neutropenia or an inappropriately low neutrophil count during bacterial infections should be evaluated for autoimmune neutropenia.
