Idiopathic neutropenia of infancy: Data from the Italian Neutropenia Registry

Piero Farruggia, Francesca Fioredda, Giuseppe Puccio, Daniela Onofrillo, Giovanna Russo, Angelica Barone, Sonia Bonanomi, Gianluca Boscarol, Andrea Finocchi, Roberta Ghilardi, Paola Giordano, Saverio Ladogana, Giuseppe Lassandro, Laura Luti, Tiziana Lanza, Rosalba Mandaglio, Nicoletta Marra, Baldassare Martire, Elena Mastrodicasa, Milena MottaLucia Dora Notarangelo, Marta Pillon, Laura Porretti, Jessica Serafinelli, Angela Trizzino, Fabio Tucci, Marinella Veltroni, Federico Verzegnassi, Ugo Ramenghi, Carlo Dufour

Research output: Contribution to journalArticlepeer-review

Abstract

Autoimmune neutropenia of infancy (AIN) is characterized by low risk of severe infection, tendency to spontaneously resolve and typically onset at ≤4–5 years of age; it is due to auto-antibodies whose detection is often difficult. In case of negativity of 4 antineutrophils autoantibody tests, after having excluded ethnic, postinfection, drug induced, or congenital neutropenia, according to the Italian guidelines the patients will be defined as affected by “idiopathic neutropenia” (IN). We describe the characteristics of 85 IN patients enrolled in the Italian neutropenia registry: they were compared with 336 children affected by AIN. The 2 groups were clinically very similar and the main differences were detection age (later in IN), length of disease (longer in IN) and, among recovered patients, age of spontaneous recovery: the median age at resolution was 2.13 years in AINs and 3.03 years in INs (P =.00002). At bivariate analysis among AIN patients earlier detection age (P =.00013), male sex (P =.000748), absence of leucopenia (P =.0045), and absence of monocytosis (P =.0419) were significantly associated with earlier recovery; in the IN group only detection age (P =.013) and absence of monocytosis (P =.0333) were significant. At multivariate analysis detection age and absence of monocytosis were independently significant (P = 6.7e-05 and 4.4e-03, respectively) in the AIN group, whereas in the IN group only detection age stayed significant (P =.013).

Original languageEnglish
Pages (from-to)216-222
JournalAmerican Journal of Hematology
Volume94
Issue number2
DOIs
Publication statusPublished - 2019

ASJC Scopus subject areas

  • Hematology

Fingerprint Dive into the research topics of 'Idiopathic neutropenia of infancy: Data from the Italian Neutropenia Registry'. Together they form a unique fingerprint.

Cite this