Idiopathic neutropenia of infancy: Data from the Italian Neutropenia Registry

Abstract

Autoimmune neutropenia of infancy (AIN) is characterized by low risk of severe infection, tendency to spontaneously resolve and typically onset at ≤4–5 years of age; it is due to auto-antibodies whose detection is often difficult. In case of negativity of 4 antineutrophils autoantibody tests, after having excluded ethnic, postinfection, drug induced, or congenital neutropenia, according to the Italian guidelines the patients will be defined as affected by “idiopathic neutropenia” (IN). We describe the characteristics of 85 IN patients enrolled in the Italian neutropenia registry: they were compared with 336 children affected by AIN. The 2 groups were clinically very similar and the main differences were detection age (later in IN), length of disease (longer in IN) and, among recovered patients, age of spontaneous recovery: the median age at resolution was 2.13 years in AINs and 3.03 years in INs (P =.00002). At bivariate analysis among AIN patients earlier detection age (P =.00013), male sex (P =.000748), absence of leucopenia (P =.0045), and absence of monocytosis (P =.0419) were significantly associated with earlier recovery; in the IN group only detection age (P =.013) and absence of monocytosis (P =.0333) were significant. At multivariate analysis detection age and absence of monocytosis were independently significant (P = 6.7e-05 and 4.4e-03, respectively) in the AIN group, whereas in the IN group only detection age stayed significant (P =.013).