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Identifying patients with neutrophil elastase (ELANE) mutations from patients with a presumptive diagnosis of autoimmune neutropenia.

Abstract：

:To differentiate severe congenital neutropenia (SCN) from autoimmune neutropenia (AIN) in patients with persistent neutropenia ≤1000/mm(3) over three months, we evaluated anti-neutrophil auto-antibodies, candidate genes of ELANA, HAX1 and GCSFR, and neutrophil elastase (NE) activity in 38 patients (21 females; average onset age 14.12 ± 2.49 months) in a primary immunodeficiency disease center between 2004 and 2011. In 30 patients, detectable anti-neutrophil auto-antibodies were HNA1a in 16 patients, HNA1c in 15, MHC Class I in 14, HNA1b in eight, MHC Class II in five, and HNA2a in three. Their average neutropenia duration was 27.04 ± 2.08 months. Of eight patients without detectable auto-antibodies, three had ELANE mutations [Ser126Pro, Arg170Phe and Cys223stop] and recurrent muco-cutaneous infections and sepsis. The patient with nonsense ELANE mutation [Cys223stop] had the lowest NE activity (16.8). Thus, patients with ELANE mutations have undetectable antibodies and more severe and younger-onset muco-cutaneous infections, prolonged healing and decreased serum NE activity that require prompt intervention.

journal_name

Immunobiology

journal_title

Immunobiology

authors

Lee WI,Chen SH,Huang JL,Jaing TH,Chung HT,Yeh KW,Chen LC,Yao TC,Hsieh MY,Lin SJ,Kuo ML

doi

10.1016/j.imbio.2012.09.001

subject

Has Abstract

pub_date

2013-05-01 00:00:00

pages

828-33

issue

5

eissn

0171-2985

issn

1878-3279

pii

S0171-2985(12)00498-6

journal_volume

218

pub_type

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