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 journalArticle

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

Fingerprint

Neutropenia
Registries
Leukopenia
Autoantibodies
Multivariate Analysis
Guidelines
Antibodies

ASJC Scopus subject areas

  • Hematology

Cite this

Idiopathic neutropenia of infancy : Data from the Italian Neutropenia Registry. / Farruggia, Piero; Fioredda, Francesca; Puccio, Giuseppe; Onofrillo, Daniela; Russo, Giovanna; Barone, Angelica; Bonanomi, Sonia; Boscarol, Gianluca; Finocchi, Andrea; Ghilardi, Roberta; Giordano, Paola; Ladogana, Saverio; Lassandro, Giuseppe; Luti, Laura; Lanza, Tiziana; Mandaglio, Rosalba; Marra, Nicoletta; Martire, Baldassare; Mastrodicasa, Elena; Motta, Milena; Notarangelo, Lucia Dora; Pillon, Marta; Porretti, Laura; Serafinelli, Jessica; Trizzino, Angela; Tucci, Fabio; Veltroni, Marinella; Verzegnassi, Federico; Ramenghi, Ugo; Dufour, Carlo.

In: American Journal of Hematology, Vol. 94, No. 2, 2019, p. 216-222.

Research output: Contribution to journalArticle

Farruggia, P, Fioredda, F, Puccio, G, Onofrillo, D, Russo, G, Barone, A, Bonanomi, S, Boscarol, G, Finocchi, A, Ghilardi, R, Giordano, P, Ladogana, S, Lassandro, G, Luti, L, Lanza, T, Mandaglio, R, Marra, N, Martire, B, Mastrodicasa, E, Motta, M, Notarangelo, LD, Pillon, M, Porretti, L, Serafinelli, J, Trizzino, A, Tucci, F, Veltroni, M, Verzegnassi, F, Ramenghi, U & Dufour, C 2019, 'Idiopathic neutropenia of infancy: Data from the Italian Neutropenia Registry', American Journal of Hematology, vol. 94, no. 2, pp. 216-222. https://doi.org/10.1002/ajh.25353
Farruggia, Piero ; Fioredda, Francesca ; Puccio, Giuseppe ; Onofrillo, Daniela ; Russo, Giovanna ; Barone, Angelica ; Bonanomi, Sonia ; Boscarol, Gianluca ; Finocchi, Andrea ; Ghilardi, Roberta ; Giordano, Paola ; Ladogana, Saverio ; Lassandro, Giuseppe ; Luti, Laura ; Lanza, Tiziana ; Mandaglio, Rosalba ; Marra, Nicoletta ; Martire, Baldassare ; Mastrodicasa, Elena ; Motta, Milena ; Notarangelo, Lucia Dora ; Pillon, Marta ; Porretti, Laura ; Serafinelli, Jessica ; Trizzino, Angela ; Tucci, Fabio ; Veltroni, Marinella ; Verzegnassi, Federico ; Ramenghi, Ugo ; Dufour, Carlo. / Idiopathic neutropenia of infancy : Data from the Italian Neutropenia Registry. In: American Journal of Hematology. 2019 ; Vol. 94, No. 2. pp. 216-222.
@article{a5fdd72f562c4378aa52376f5badb955,
title = "Idiopathic neutropenia of infancy: Data from the Italian Neutropenia Registry",
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).",
author = "Piero Farruggia and Francesca Fioredda and Giuseppe Puccio and Daniela Onofrillo and Giovanna Russo and Angelica Barone and Sonia Bonanomi and Gianluca Boscarol and Andrea Finocchi and Roberta Ghilardi and Paola Giordano and Saverio Ladogana and Giuseppe Lassandro and Laura Luti and Tiziana Lanza and Rosalba Mandaglio and Nicoletta Marra and Baldassare Martire and Elena Mastrodicasa and Milena Motta and Notarangelo, {Lucia Dora} and Marta Pillon and Laura Porretti and Jessica Serafinelli and Angela Trizzino and Fabio Tucci and Marinella Veltroni and Federico Verzegnassi and Ugo Ramenghi and Carlo Dufour",
year = "2019",
doi = "10.1002/ajh.25353",
language = "English",
volume = "94",
pages = "216--222",
journal = "American Journal of Hematology",
issn = "0361-8609",
publisher = "Wiley-Liss Inc.",
number = "2",

}

TY - JOUR

T1 - Idiopathic neutropenia of infancy

T2 - Data from the Italian Neutropenia Registry

AU - Farruggia, Piero

AU - Fioredda, Francesca

AU - Puccio, Giuseppe

AU - Onofrillo, Daniela

AU - Russo, Giovanna

AU - Barone, Angelica

AU - Bonanomi, Sonia

AU - Boscarol, Gianluca

AU - Finocchi, Andrea

AU - Ghilardi, Roberta

AU - Giordano, Paola

AU - Ladogana, Saverio

AU - Lassandro, Giuseppe

AU - Luti, Laura

AU - Lanza, Tiziana

AU - Mandaglio, Rosalba

AU - Marra, Nicoletta

AU - Martire, Baldassare

AU - Mastrodicasa, Elena

AU - Motta, Milena

AU - Notarangelo, Lucia Dora

AU - Pillon, Marta

AU - Porretti, Laura

AU - Serafinelli, Jessica

AU - Trizzino, Angela

AU - Tucci, Fabio

AU - Veltroni, Marinella

AU - Verzegnassi, Federico

AU - Ramenghi, Ugo

AU - Dufour, Carlo

PY - 2019

Y1 - 2019

N2 - 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).

AB - 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).

UR - http://www.scopus.com/inward/record.url?scp=85058693958&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85058693958&partnerID=8YFLogxK

U2 - 10.1002/ajh.25353

DO - 10.1002/ajh.25353

M3 - Article

AN - SCOPUS:85058693958

VL - 94

SP - 216

EP - 222

JO - American Journal of Hematology

JF - American Journal of Hematology

SN - 0361-8609

IS - 2

ER -