Diagnosis and management of primary autoimmune neutropenia in children: insights for clinicians

Farruggia, Piero; Dufour, Carlo

doi:10.1177/2040620714556642

Cited by 46 publications

(50 citation statements)

References 81 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…There is also overlap of patients with the diagnosis of CIN and “autoimmune neutropenia (AIN),” because it is difficult to accurately detect circulating antibodies directed toward antigens present on the surface of neutrophils, and clinical interpretation of the anti-neutrophil antibody test result is also difficult. [6, 7] This report summarizes the recent literature on CIN and AIN and presents observations from the Severe Chronic Neutropenia International Registry (SCNIR) on these conditions. [8] Several reviews and commentaries on the diagnosis and treatment of chronic neutropenia in children and adults have been published recently.…”

Section: Introductionmentioning

confidence: 99%

An update on the diagnosis and treatment of chronic idiopathic neutropenia

Dale¹,

Bolyard

2017

Current Opinion in Hematology

View full text Add to dashboard Cite

Purpose of Review Neutropenia lasting for at least for 3 months and not attributable to drugs or a specific genetic, infectious, inflammatory, autoimmune or malignant cause is called chronic idiopathic neutropenia. (CIN) CIN and autoimmune neutropenia (AIN) are very similar and overlapping conditions. The clinical consequences depend upon the severity of neutropenia, but it is not considered a premalignant condition. Recent findings Long-term observational studies in children indicate that the disease often lasts for 3 to 5 years in children, then spontaneously remits, but it rarely remits in adult cases. The value of anti-neutrophil antibody testing in both children and adults is uncertain. Most recent data suggest that CIN and AIN are immune mediated diseases, but there are no new clinical or genetic tests to aid in diagnosis. Treatment with granulocyte colony stimulating factor (G-CSF) is effective to increase blood neutrophils in almost all cases; this treatment is reserved, however for patients with both neutropenia and evidence of recurrent fevers, inflammatory symptoms and infections. There is little or no evidence to indicate that G-CSF treatment predisposes to myeloid malignancies in this population. Summary It is important to recognize CIN and AIN, the most common causes of chronic neutropenia in both children and adults. If the neutropenia is not severe, i.e. > 0.5 × 109/L, most patients can be observed and not treated prophylactically with antibiotics or a growth factor. When neutropenia is severe treatment with G-CSF is often beneficial.

show abstract

Section: Introductionmentioning

confidence: 99%

An update on the diagnosis and treatment of chronic idiopathic neutropenia

Dale¹,

Bolyard

2017

Current Opinion in Hematology

View full text Add to dashboard Cite

show abstract

“…It is more commonly observed in girls and children younger than two years old [2]. In most cases, the course of the disease is benign and self-limited [6].…”

Section: Discussionmentioning

confidence: 99%

“…Many authors suggest prophylactic antibiotic (Trimethoprim/ sulfamethoxazole) to reduce infection frequency in patients with autoimmune disease. It is also suggested that G-CSF be started in case of serious infections [6].…”

Section: Discussionmentioning

confidence: 99%

Autoimmune Transient Neutropenia: A Case Report

Aparecida¹,

Frantz²,

Scheidemantel³

2017

Clin Pediatr Dermatol

View full text Add to dashboard Cite

“…AIN typically presents within the first two years of life with the average age of onset between 7 and 9 months . The neutropenia is often detected during an acute febrile illness, which persists upon follow‐up testing after resolution of the infection, or incidentally when a complete blood count is obtained for another reason . AIN may be subdivided into two categories: primary, in which neutropenia is an isolated finding, or secondary, in which the AIN is a manifestation of an underlying disorder (e.g., immune dysregulation and malignancy)…”

Section: Introductionmentioning

confidence: 99%

“…[5][6][7] The neutropenia is often detected during an acute febrile illness, which persists upon follow-up testing after resolution of the infection, or incidentally when a complete blood count is obtained for another reason. 5,7,8 AIN may be subdivided into two categories: primary, in which neutropenia is an isolated finding, or secondary, in which the AIN is a manifestation of an underlying disorder (e.g., immune dysregulation and malignancy). 1 Neutropenia in AIN is a result of peripheral destruction of neutrophils due to autoantibody production against antigens on the neutrophil surface, most commonly human neutrophil antigen 1 (HNA-1) on the neutrophil immunoglobulin receptor Fc RIIIb.…”

Section: Introductionmentioning

confidence: 99%

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

Kirk

Grimes

Shelke

et al. 2019

Pediatric Blood & Cancer

View full text Add to dashboard Cite

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.

show abstract

Diagnosis and management of primary autoimmune neutropenia in children: insights for clinicians

Cited by 46 publications

References 81 publications

An update on the diagnosis and treatment of chronic idiopathic neutropenia

An update on the diagnosis and treatment of chronic idiopathic neutropenia

Autoimmune Transient Neutropenia: A Case Report

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

Contact Info

Product

Resources

About