TY - JOUR T1 - Idiopathic neutropenia of infancy: Data from the Italian Neutropenia Registry. JF - Am J Hematol Y1 - 2019 A1 - Farruggia, Piero A1 - Fioredda, Francesca A1 - Puccio, Giuseppe A1 - Onofrillo, Daniela A1 - Russo, Giovanna A1 - Barone, Angelica A1 - Bonanomi, Sonia A1 - Boscarol, Gianluca A1 - Finocchi, Andrea A1 - Ghilardi, Roberta A1 - Giordano, Paola A1 - Ladogana, Saverio A1 - Lassandro, Giuseppe A1 - Luti, Laura A1 - Lanza, Tiziana A1 - Mandaglio, Rosalba A1 - Marra, Nicoletta A1 - Martire, Baldassare A1 - Mastrodicasa, Elena A1 - Motta, Milena A1 - Notarangelo, Lucia Dora A1 - Pillon, Marta A1 - Porretti, Laura A1 - Serafinelli, Jessica A1 - Trizzino, Angela A1 - Tucci, Fabio A1 - Veltroni, Marinella A1 - Verzegnassi, Federico A1 - Ramenghi, Ugo A1 - Dufour, Carlo 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).

VL - 94 IS - 2 U1 - http://www.ncbi.nlm.nih.gov/pubmed/30456824?dopt=Abstract ER -