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

Abstract

Background Autoimmune neutropenia (AIN) is a common cause of chronic neutropenia in childhood. Despite an expected benign clinical course, many patients undergo extensive evaluation. Data on healthcare utilization and rates of bloodstream infections in young patients with AIN are limited. Methods All patients with a diagnosis code of leukopenia, neutropenia, or AIN followed within the outpatient hematology clinic of a single institution from 2014 to 2016 were identified. Patients aged ≤5 years with absolute neutrophil count (ANC) ≤500/µL persisting for ≥3 months, a clinical diagnosis of AIN, and documented resolution of neutropenia were included. Data on clinical management, including infectious outcomes and emergency center (EC) encounters, were collected. Results Forty‐three patients with AIN (18 male [42%], median age at diagnosis 12 months) met eligibility criteria. Children were followed by hematology for a median duration of 18 (range, 2‐85) months. Diagnostic evaluations were variable. Thirty patients (70%) had ≥ 1 EC encounters for evaluation of isolated fever with a total of 113 EC encounters for the overall cohort. Patients with ANC < 500/µL and isolated fever were admitted for observation, which resulted in 24 hospitalizations in 16 patients. Of 138 blood cultures drawn, two were positive, both later determined to be contaminants. Conclusion At a large tertiary care center, no bloodstream infections were identified in a cohort of 43 children with AIN presenting to the EC for assessment of fever. A less‐intensive, more cost‐effective management paradigm, which continues to prioritize patient safety, among young children with AIN is needed.

The ASH‐ASPHO Choosing Wisely Campaign: 5 hematologic tests and treatments to question

“…There are limited data regarding the use of subcutaneous G‐CSF in children with AIN. Two recent studies report administering G‐CSF in 7.5% to 16% of children with AIN, mainly as on‐demand regimens in the event of recurrent infections or before planned invasive procedures 51,52 . However, neither study demonstrated clear benefits in reducing infection rates, including pathogenic bloodstream infections 51,52 .…”

“…Two recent studies report administering G‐CSF in 7.5% to 16% of children with AIN, mainly as on‐demand regimens in the event of recurrent infections or before planned invasive procedures 51,52 . However, neither study demonstrated clear benefits in reducing infection rates, including pathogenic bloodstream infections 51,52 . Therefore, in children with asymptomatic AIN, there is insufficient evidence to support the routine use of G‐CSF as a prophylaxis strategy for improving health outcomes 48,49,53,54 .…”

The blood protein hCAP‐18 in neutropenia: An 18‐month experience of a new ELISA for clinical use

“…Autoimmune neutropenia is the most common diagnosis for paediatric patients with chronic neutropenia, and the question of cost effectiveness in management of patients with AIN without compromising patient safety has been raised. 11 The general practice relying on neutrophil-specific antibodies lacks in sensitivity but also in specificity as cases of SCN may be initially judged to be AIN following positive antibody results, and thus delaying further investigation. Additional specific diagnostic and clinical criteria for detecting the severe cases and excluding the benign cases from more elaborate testing, such as the present ELISA, would allow for significant cost reduction.…”

“…Medical and family history as well as complete blood counts at the initial medical examination might be sufficient to establish a diagnosis 9 . However, it is clear from many reports that differential diagnosis may be challenging, wherefore extensive and invasive investigations are often performed also in cases with benign neutropenia, which cause both patient stress and drain limited resources 10,11 . Additional blood markers of severe disease that easily can be applied in clinical workup are warranted.…”

Prevalência, perfil clínico e evolutivo de neutropenias crônicas autoimunes em crianças e adolescentes acompanhados em ambulatórios terciários de hematologia, imunologia e reumatologia

“…Após o diagnóstico, a média de infecções caiu de 1,2 por ano para 0,2 por ano, reforçando a importância da identificação precoce das causas de neutropenia e a instituição do tratamento específico o mais rápido possível. Kirk et al, identificaram a ocorrência de infecções em 77% dos pacientes com NAI, sendo as mais ocorrentes otite média aguda, infecções cutâneas e pneumonia (Kirk et al, 2020). Farruggia et al, encontraram maior frequência de infecções nos pacientes com NAI de causa secundária (40%) quando comparados com os pacientes de NAI primária (11.8%) (Farruggia et al, 2017).…”