Infectious Complications In Children With Severe Congenital, Autoimmune Or Idiopathic Neutropenia

Abstract

We describe the incidence and characteristics of infections in children with severe congenital neutropenia (SCN), autoimmune neutropenia (AN) and idiopathic neutropenia (IN). Data extracted from the Italian Neutropenia Registry on 73 patients with 108 episodes of infections were collected from 2000 to 2009. All SCN patients with SCN and one third of AN and IN experienced at least 1 infectious episode, equating to 5.7 infections/patient in SCN and approximately 0.6 in AN and IN. The rate of infections before diagnosis of neutropenia was 6.35/1000 patient-days at risk in SCN, 0.48 in AN and 0.71 in IN (P < 0.001) and significantly decreased after diagnosis. Skin and subcutaneous abscesses and cellulitis were the most frequent types of infection encountered, followed by pneumonia. Infections are an important clinical issue in the management of neutropenic patients, even in those considered at lower risk.

The epidemiology, clinical course and outcome of febrile cytopenia in children

“…Only two cases with autoimmune neutropenia developed cellulitis and perianal abscess, respectively, and recovered after antibiotic treatment. The findings of our study are in agreement with previous reports (1,3,15,29). It seems that the adequacy of bone marrow reserve, along with a satisfactory neutrophil function, are the most critical determinants of propensity to infection (30).…”

Neutropenia During Tocilizumab Treatment Is Not Associated with Infection Risk in Systemic or Polyarticular-course Juvenile Idiopathic Arthritis

“…It is worth noting that we evaluated the occurrence of infections during grade ≥ 3 neutropenia over a duration of 5.5 years in patients with sJIA and 2.1 years in patients with pcJIA. In an observational study in patients with severe congenital neutropenia, the rate of serious infection was 232/100 PY before the administration of G-CSF and 38/100 PY after diagnosis and the administration of G-CSF 40 .…”

“…It is possible that blockade of IL-6 may modulate the circulating pool of neutrophils by shifting them from the circulating to the marginated pools 21,41 , or by increasing their transit time through the bone marrow, or both 21,42 . Moreover, after TCZ administration, neutrophils are fully functional and show no impairment in their antibacterial functions 40 .…”

The cost of a “benign” condition: Healthcare utilization and infectious outcomes in young children with primary autoimmune neutropenia

“…Multiple retrospective reviews describe a relatively low risk of serious infection in children with AIN despite the development of moderate to severe neutropenia. 3,5,16,17 Common childhood infections such as upper respiratory infections, acute otitis media or skin infections, such as cellulitis, may be present. 3 In patients with prolonged, severe neutropenia, mouth sores may occur.…”