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 language | English |
|---|---|
| Pages (from-to) | 216-222 |
| Journal | American Journal of Hematology |
| Volume | 94 |
| Issue number | 2 |
| DOIs | |
| Publication status | Published - 2019 |
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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 journal › Article
}
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).
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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 -