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Diagnosis of autoimmune neutropenia in a 10-month-old boy – a case report
Central European Journal of Immunology ( IF 1.5 ) Pub Date : 2021-03-11 , DOI: 10.5114/ceji.2021.104327
Katarzyna Zdanowicz , Urszula Daniluk , Elena Jewsiejenko , Milena Krasnodębska , Radosław Motkowski , Dariusz Lebensztejn

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.

中文翻译:

10个月大男孩自身免疫性中性粒细胞减少的诊断-病例报告

先天性或获得性中性粒细胞减少症与骨髓中粒细胞产生受损或抗体破坏增加有关。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出现有关。AIN是婴幼儿中性粒细胞减少的最常见原因。但是,它的发生率很低。抗中性粒细胞抗体的检测是确诊AIN的重要步骤,尽管由于滴度低和亲和力差而难以检测。在鉴别诊断中,应考虑中性粒细胞减少的另一原因,例如药物引起的机制,病毒感染,自身免疫和代谢疾病,血液学状况或免疫缺陷综合症。尽管AIN病情良好,但仍可能发生严重的感染并发症。在随访的24个月中,有95%的患者观察到中性白细胞减少症的自发缓解。我们介绍了一个病例,该病例为一个10个月大的男孩,由于自身免疫性中性粒细胞减少症形成皮肤脓肿,因此在我们部门被诊断为耳聋,心脏缺陷和Morgagni-Larrey疝。
更新日期:2021-04-18
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