Pediatric Hematology and Oncology Unit, A.R.N.A.S. Ospedale Civico, Palermo, Italy.
Clinical and Experimental Unit G. Gaslini Children's Hospital, Genoa, Italy.
Am J Hematol. 2019 Feb;94(2):216-222. doi: 10.1002/ajh.25353. Epub 2018 Dec 18.
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).
婴儿自身免疫性中性粒细胞减少症 (AIN) 的特征为严重感染风险低、有自发缓解倾向且发病年龄通常≤4-5 岁；该病由自身抗体引起，其检测通常较为困难。若 4 项抗中性粒细胞自身抗体检测均为阴性，在排除了种族、感染后、药物诱导或先天性中性粒细胞减少症后，根据意大利指南，患者将被定义为患有“特发性中性粒细胞减少症” (IN)。我们描述了意大利中性粒细胞减少症登记处纳入的 85 例 IN 患者的特征：将其与 336 例 AIN 患儿进行了比较。这两组在临床上非常相似，主要区别在于发病年龄（IN 组较晚）、疾病持续时间（IN 组较长）和已恢复患者中自发缓解的年龄：AIN 组的中位缓解年龄为 2.13 岁，IN 组为 3.03 岁（P=.00002）。在 AIN 患者中，二变量分析显示，较早的检测年龄（P=.00013）、男性（P=.000748）、无白细胞减少症（P=.0045）和无单核细胞增多症（P=.0419）与更早的恢复显著相关；而在 IN 组中，仅检测年龄（P=.013）和无单核细胞增多症（P=.0333）有显著意义。多变量分析显示，在 AIN 组中，检测年龄和无单核细胞增多症是独立的显著因素（P 值分别为 6.7e-05 和 4.4e-03），而在 IN 组中，仅检测年龄仍有显著意义（P=.013）。
