Jinca Cristian, Serban Margit, Ursu Emilia, Munteanu Andrei, Arghirescu Smaranda
Department of Pediatrics, 'Victor Babes' University of Medicine and Pharmacy, 300041 Timisoara, Romania.
Department of Onco-Hematology, 'Louis Turcanu' Emergency Hospital for Children, 300011 Timisoara, Romania.
Exp Ther Med. 2021 Mar;21(3):280. doi: 10.3892/etm.2021.9711. Epub 2021 Jan 25.
Neutropenia is commonly diagnosed in pediatric clinics. Due to the special vulnerability of neutropenic patients, the assessment of the etiopathogenic background of neutropenia is mandatory. In this retrospective cross-sectional cohort study, we aimed to establish the status of primary autoimmune neutropenia (AIN) from the point of view of its clinical and biological features and its outcome in a cohort of pediatric patients. We recorded all of the 3,488 cases consecutively admitted to our hospital for different diagnoses but presenting neutropenia, during a period of 3 years (January 2016 to December 2018). We had to exclude 224 patients from the analysis due to incomplete data. Our study focused on patients with AIN or chronic benign neutropenia of infancy and childhood. In these patients, a granulocyte antibody screening by granulocyte immunofluorescence test (GIFT) and the granulocyte agglutination test (GAT) were performed. Regarding their pathogenic background, 0.1% of the patients presenting neutropenia were congenital forms, the rest being acquired forms. Primary AIN was encountered in 18 cases, representing approximately 0.5%. The median age at onset for primary AIN was 7.5 months. Male/female ratio in AIN was 1.94. In 72% of the patients with AIN, neutropenia was severe during the course of disease. In 3 patients, both GIFT and GAT were positive and in 8 patients, only GIFT was positive. For the remaining 7 patients (39%), both GIFT and GAT revealed negative results. 50% of the patients needed hospitalization, but only 3 patients presented severe infections. On-demand G-CSF was administered in 22% of the patients. Our study provides insight with regard to neutropenia, showing the high frequency and etiological diversity in childhood. Primary AIN is usually diagnosed by exclusion of the other causes of neutropenia. GIFT and GAT are useful, but rarely available diagnostic tools for the confirmation of primary AIN.
中性粒细胞减少症在儿科诊所中很常见。由于中性粒细胞减少症患者具有特殊的易感性，因此必须评估中性粒细胞减少症的病因背景。在这项回顾性横断面队列研究中，我们旨在从临床和生物学特征及其在一组儿科患者中的结局的角度，确定原发性自身免疫性中性粒细胞减少症（AIN）的状况。我们记录了在3年期间（2016年1月至2018年12月）因不同诊断而连续入住我院但出现中性粒细胞减少症的所有3488例病例。由于数据不完整，我们不得不将224例患者排除在分析之外。我们的研究重点是患有AIN或婴儿期和儿童期慢性良性中性粒细胞减少症的患者。在这些患者中，通过粒细胞免疫荧光试验（GIFT）和粒细胞凝集试验（GAT）进行粒细胞抗体筛查。关于其致病背景，出现中性粒细胞减少症的患者中有0.1%为先天性形式，其余为后天获得性形式。原发性AIN有18例，约占0.5%。原发性AIN的发病中位年龄为7.5个月。AIN中的男女比例为1.94。在72%的AIN患者中，疾病过程中中性粒细胞减少症很严重。在3例患者中，GIFT和GAT均为阳性，在8例患者中，仅GIFT为阳性。对于其余7例患者（39%），GIFT和GAT均显示阴性结果。50%的患者需要住院治疗，但只有3例患者出现严重感染。22%的患者按需使用了粒细胞集落刺激因子（G-CSF）。我们的研究提供了关于中性粒细胞减少症的见解，显示了儿童期的高发病率和病因多样性。原发性AIN通常通过排除中性粒细胞减少症的其他原因来诊断。GIFT和GAT是有用的，但很少有用于确诊原发性AIN的诊断工具。
