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病例系列:先天性肠道病毒感染相关噬血细胞性淋巴组织细胞增生症及随后的中性粒细胞减少症

Case series: Congenital enterovirus infection-associated haemophagocytic lymphohistiocytosis and subsequent neutropaenia.

作者信息

Penner Justin, Burns James E, Breuer Judith, Gilmour Kimberly C, Bamford Alasdair, Rao Anupama

机构信息

Department of Paediatric Infectious Diseases Great Ormond Street Hospital London UK.

UCL Centre for Clinical Research in Infection and Sexual Health Institute for Global Health London UK.

出版信息

EJHaem. 2024 Jun 18;5(4):784-788. doi: 10.1002/jha2.931. eCollection 2024 Aug.

Abstract

Congenital enterovirus infection can be associated with a pro-inflammatory state triggering haemophagocytic lymphohistiocytosis (HLH). Enteroviruses are also known to cause transient neutropenia in healthy children. Two infants presented with temperature instability, lethargy, thrombocytopaenia, hepatosplenomegaly and evidence of hyperinflammation in the setting of perinatal maternal rash and household contacts with gastrointestinal symptoms. Whilst HLH was successfully treated in both, protracted neutropenia persisted. Immune dysregulation with enterovirus in the neonatal period can provoke the generation of autoantibodies to hematologic cells giving rise to conditions such as autoimmune neutropenia. Sustained neutropaenia, after resolution of secondary infectious forms of HLH, requires investigation for underlying aetiologies.

摘要

先天性肠道病毒感染可能与促炎状态相关,从而引发噬血细胞性淋巴组织细胞增生症(HLH)。肠道病毒还已知会在健康儿童中引起短暂性中性粒细胞减少。两名婴儿出现体温不稳定、嗜睡、血小板减少、肝脾肿大,且在围产期母亲出疹以及有胃肠道症状的家庭接触史的情况下有炎症反应加剧的迹象。虽然两名婴儿的HLH均得到成功治疗,但持续性中性粒细胞减少仍持续存在。新生儿期肠道病毒引起的免疫失调可引发针对血液细胞的自身抗体生成,从而导致自身免疫性中性粒细胞减少等病症。在继发性感染性HLH消退后,持续性中性粒细胞减少需要对潜在病因进行调查。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/796e/11327720/7463a6eecc92/JHA2-5-784-g001.jpg

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