Etiology, clinical outcome, and laboratory features in children with neutropenia: analysis of 104 cases.

BACKGROUND

Neutropenia is not uncommon in childhood. The aim of our study was to analyze the underlying causes of neutropenia and to evaluate its clinical significance in a series of children referred to our center.

METHODS

One hundred and four consecutive children with neutropenia were enrolled in this study. Clinical and laboratory features were analyzed.

RESULTS

The majority of patients (63.5%) showed chronic neutropenia. Among all chronic forms, the most frequent was chronic idiopathic neutropenia (CIN), followed by autoimmune neutropenia (AIN). Congenital neutropenia was identified in 6 patients. Acute neutropenia was mainly due to infections. Overall, at the time of first detection, neutropenia was more frequently severe or moderate. One-third of our patients who presented with severe neutropenia were ultimately diagnosed with a post-infectious acute form. Conversely, nearly half patients with CIN, AIN, or congenital neutropenia showed moderate/mild neutropenia at onset. Among patients with AIN and CIN, nearly half recovered between 7 months and 46 months and approximately one-fourth experienced infectious episodes during follow-up. No significant difference was noticed in terms of mean ANC between patients with and without remission, neither between patients with and without infections.

CONCLUSIONS

Our study confirms the great etiological heterogeneity of neutropenia in children. We could not demonstrate a correlation between ANC level at onset and the underlying disorder, nor a correlation between mean ANC and duration of neutropenia or infectious episodes during follow-up. Neutropenia remains a disease of concern to pediatricians, requiring several laboratory investigations, prolonged follow-up, and, in few cases, advanced molecular methods.