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Significance of anti-neutrophil antibody in chronic benign neutropenia in Chinese children
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  • Shau yin Ha,
  • Sophie Lai,
  • Wing Shan See,
  • Jaime Duque,
  • Daniel Ka Leung Cheuk,
  • Jenny Ho,
  • Stephen Cheung ,
  • Patrick Chu,
  • Pamela PW Lee,
  • Janette SY Kwok
Shau yin Ha
Queen Mary Hospital

Corresponding Author:[email protected]

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Sophie Lai
Queen Mary Hospital
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Wing Shan See
Queen Mary Hospital
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Jaime Duque
The University of Hong Kong
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Daniel Ka Leung Cheuk
Hong Kong Children's Hospital
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Jenny Ho
Division of Transplantation and Immunogenetics, Department of Pathology & Clinical Biochemistry, Queen Mary Hospital
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Stephen Cheung
Division of Transplantation and Immunogenetics, Department of Pathology & Clinical Biochemistry, Queen Mary Hospital
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Patrick Chu
Division of Transplantation and Immunogenetics, Department of Pathology & Clinical Biochemistry, Queen Mary Hospital
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Pamela PW Lee
The University of Hong Kong
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Janette SY Kwok
Division of Transplantation and Immunogenetics, Department of Pathology & Clinical Biochemistry, Queen Mary Hospital
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Abstract

Background: Chronic benign neutropenia (CBN) and autoimmune neutropenia (AIN) are notoriously difficult to differentiate in children owing to their indistinguishable clinical course and varying availability and accuracy in the methods of anti-neutrophil antibody detection. This study aims to investigate whether the presence of anti-neutrophil antibody has implications on the disease course in Chinese children with AIN, as well as evaluating the various methods including LABScreen MULTI, granulocyte agglutination test (GAT) and granulocyte immunofluorescence test (GIFT) in anti-neutrophil antibody detection. Procedure: Chinese children under 18 years of age with neutropenia ≤ 1.5 x 109/L lasting 6 months or more in our single center were recruited into the study between 2016 to 2018. Patients with secondary causes of neutropenia were excluded. Blood for anti-neutrophil antibody and genotyping was taken once at the time of recruitment and subsequently when neutropenia recovered to ≥ 1.5 x 109/L. A combination of two in-house methods including GIFT, GAT and commercial kit LABScreenTM multipanel were used for the detection of antibodies. The age of onset, age of recovery, duration of neutropenia, gender, serial neutrophil counts, incidence of invasive infection, use of G-CSF were examined. Results: Using combined testing methods, anti-neutrophil antibody was detected in 30.8 % of patients and positivity was associated with more severe neutropenia, higher likelihood of infection and slower and later recovery compared to those without antibodies. Conclusions: The presence of anti-neutrophil antibody was useful in predicting the clinical course of patients with AIN. The use of combined testing methods increased the detection rate.