Paediatric autoimmune neutropaenia

Abstract

Neutropaenia is defined as an absolute neutrophil count (ANC) of less than 1500 cells/µL and is often divided into mild (ANC: 1000 to 1500 cells/µL), moderate (ANC: 500 to 1000 cells/µL) and severe (ANC: >500 cells/µL) neutropaenia. Autoimmune neutropaenia is arguably one of the most clinically relevant forms of neutropaenia and is defined by an ANC of <500 cells/µL alongside the confirmed presence of anti-neutrophil antibodies. It is a rare subset of neutropaenia boasting an incidence of just 1 in 100 000 infants and is thought to be caused as a result of a 'surveillance escape event' of the immune system. This case report identifies key issues in the diagnosis and monitoring of paediatric patients with recurrent neutropaenia and reviews current literature relating to its management in hospital and community settings.

Keywords: haematology (incl blood transfusion); infant health; paediatrics.