Institute of Hematology and Transfusion Medicine, Medical College and Hospital, Kolkata, West Bengal, India. Correspondence to: Dr Sukrita Bhattacharjee, Department of Hematology, Institute of Hematology and Transfusion Medicine, Medical College and Hospital, Kolkata, India.
Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India.
Indian Pediatr. 2020 Sep 15;57(9):827-833. doi: 10.1007/s13312-020-1962-z. Epub 2020 Jun 24.
Preliminary data on coexistence of secondary hemophagocytic lymphohistiocytosis syndrome (HLH) and disseminated intravascular coagulation (DIC) in critically ill children with novel coronavirus disease (COVID-19) are emerging. Herein, we summarize the available literature and fill-in the gaps in this regard.
We have performed a literature search for articles in PubMed, EMBASE and Google Scholar databases till May 12, 2020, with following keywords: "COVID-19", "SARS-CoV-2", "HLH", "HScore", "coagulopathy", "D-dimer", "cytokine storm", "children" and "pediatrics" with interposition of Boolean operator "AND".
Children presenting with moderate-severe COVID-19 and Kawasaki disease shock-like syndrome exhibit peripheral blood picture analogous to HLH. HScore, a validated tool to diagnose HLH, has been suggested to screen severe COVID-19 patients for cytokine storm. However, HScore faces certain limitations in this scenario. It may be more pragmatic to use 'high D-dimer' (> 3 µg/mL) instead of 'low fibrinogen' to facilitate early detection of cytokine storm. COVID-19 associated coagulopathy resembles hypercoagulable form of DIC with bleeding being rarely reported. Although the International Society on Thrombosis and Haemostasis (ISTH) interim guidance recommends low molecular weight heparin in all hospitalized patients, data is lacking in population below 14 years of age. However, in the presence of life-threatening thromboembolic event or symptomatic acro-ischemia, unfractionated heparin (UFH) should be used with caution.
HScore can be used as a complement to clinical decision for initiating immunosuppression. Children with moderate-to-severe COVID-19, especially those with documented thrombocytopenia or chilblains, should be regularly monitored for coagulopathy.
新型冠状病毒病（COVID-19）危重症患儿并发噬血细胞性淋巴组织细胞增生症（HLH）和弥漫性血管内凝血（DIC）的初步数据正在出现。在此，我们对文献进行综述，并填补这方面的空白。
我们在 PubMed、EMBASE 和 Google Scholar 数据库中进行了文献检索，检索时间截至 2020 年 5 月 12 日，使用的关键词如下：“COVID-19”、“SARS-CoV-2”、“HLH”、“HScore”、“凝血障碍”、“D-二聚体”、“细胞因子风暴”、“儿童”和“儿科学”，并使用布尔运算符“AND”进行干预。
患有中度至重度 COVID-19 和川崎病休克样综合征的儿童表现出类似于 HLH 的外周血象。HScore 是一种用于诊断 HLH 的验证工具，已被建议用于筛选重症 COVID-19 患者的细胞因子风暴。然而，HScore 在这种情况下存在一定的局限性。使用“高 D-二聚体”（>3μg/ml）代替“低纤维蛋白原”可能更具实用性，以方便早期检测细胞因子风暴。COVID-19 相关的凝血障碍类似于高凝型 DIC，很少有出血报道。尽管国际血栓与止血学会（ISTH）临时指南建议所有住院患者使用低分子肝素，但在 14 岁以下人群中缺乏数据。然而，在存在危及生命的血栓栓塞事件或症状性肢端缺血时，应谨慎使用普通肝素（UFH）。
HScore 可作为启动免疫抑制治疗的临床决策的补充。中重度 COVID-19 的患儿，尤其是有血小板减少或冻疮病史的患儿，应定期监测凝血障碍。
