Zdanowicz Katarzyna, Daniluk Urszula, Jewsiejenko Elena, Krasnodębska Milena, Motkowski Radosław, Lebensztejn Dariusz Marek
Department of Pediatrics, Gastroenterology, Hepatology, Nutrition and Allergology, Medical University of Bialystok, Poland.
Department of Pediatrics, Rheumatology, Immunology and Metabolic Bone Diseases, Medical University of Bialystok, Poland.
Cent Eur J Immunol. 2021;46(1):118-120. doi: 10.5114/ceji.2021.104327. Epub 2021 Mar 11.
Neutropenia, congenital or acquired, is related to impaired granulocyte production in the bone marrow or increased destruction by antibodies. Autoimmune neutropenia of infancy (AIN) is associated with the occurrence of antineutrophil antibodies. AIN is the most common cause of neutropenia in infants and young children. However, its incidence is low. Detection of anti-neutrophil antibodies is an important step in confirming the diagnosis of AIN, although their detection is difficult due to low titer and poor avidity. In differential diagnosis, another cause of neutropenia should be considered, such as a drug-induced mechanism, viral infection, autoimmune and metabolic disease, hematological conditions or immune deficiency syndromes. Despite the benign course of AIN, serious infectious complications can occur. Spontaneous remission of neutropenia was observed in 95% of patients during 24 months of follow-up. We present a case of a 10-month-old boy with deafness, heart defect and Morgagni-Larrey hernia diagnosed in our department because of formation of a skin abscess due to autoimmune neutropenia.
先天性或后天性中性粒细胞减少症与骨髓中粒细胞生成受损或抗体介导的破坏增加有关。婴儿自身免疫性中性粒细胞减少症（AIN）与抗中性粒细胞抗体的产生有关。AIN是婴幼儿中性粒细胞减少症最常见的原因。然而，其发病率较低。抗中性粒细胞抗体的检测是确诊AIN的重要步骤，尽管由于其滴度低和亲和力差，检测较为困难。在鉴别诊断中，应考虑中性粒细胞减少症的其他原因，如药物诱导机制、病毒感染、自身免疫和代谢性疾病、血液系统疾病或免疫缺陷综合征。尽管AIN病程良性，但仍可能发生严重的感染并发症。在24个月的随访中，95%的患者中性粒细胞减少症出现自发缓解。我们报告了一例10个月大的男孩，因自身免疫性中性粒细胞减少症导致皮肤脓肿，在我院被诊断为患有耳聋、心脏缺陷和莫尔加尼-拉里疝。
