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COVID-19 Associated Hemophagocytic Lymphohistiocytosis and Coagulopathy: Targeting the Duumvirate.
Indian Pediatr. 2020 Sep 15; 57(9):827-833.IP

Abstract

CONTEXT

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.

EVIDENCE ACQUISITION

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".

RESULTS

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.

CONCLUSIONS

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.

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Authors+Show Affiliations

Bhattacharjee S
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. skrtbhttchry@gmail.com.
Banerjee M
Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India.
Pal R
Post Graduate Institute of Medical Education and Research, Chandigarh, India.

MeSH

BetacoronavirusBlood Coagulation DisordersCOVID-19Coronavirus InfectionsHumansLymphohistiocytosis, HemophagocyticPandemicsPneumonia, ViralSARS-CoV-2

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

32583809

Citation

Bhattacharjee, Sukrita, et al. "COVID-19 Associated Hemophagocytic Lymphohistiocytosis and Coagulopathy: Targeting the Duumvirate." Indian Pediatrics, vol. 57, no. 9, 2020, pp. 827-833.
Bhattacharjee S, Banerjee M, Pal R. COVID-19 Associated Hemophagocytic Lymphohistiocytosis and Coagulopathy: Targeting the Duumvirate. Indian Pediatr. 2020;57(9):827-833.
Bhattacharjee, S., Banerjee, M., & Pal, R. (2020). COVID-19 Associated Hemophagocytic Lymphohistiocytosis and Coagulopathy: Targeting the Duumvirate. Indian Pediatrics, 57(9), 827-833.
Bhattacharjee S, Banerjee M, Pal R. COVID-19 Associated Hemophagocytic Lymphohistiocytosis and Coagulopathy: Targeting the Duumvirate. Indian Pediatr. 2020 Sep 15;57(9):827-833. PubMed PMID: 32583809.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - COVID-19 Associated Hemophagocytic Lymphohistiocytosis and Coagulopathy: Targeting the Duumvirate. AU - Bhattacharjee,Sukrita, AU - Banerjee,Mainak, AU - Pal,Rimesh, Y1 - 2020/06/24/ PY - 2020/6/26/pubmed PY - 2020/10/9/medline PY - 2020/6/26/entrez SP - 827 EP - 833 JF - Indian pediatrics JO - Indian Pediatr VL - 57 IS - 9 N2 - CONTEXT: 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. EVIDENCE ACQUISITION: 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". RESULTS: 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. CONCLUSIONS: 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. SN - 0974-7559 UR - https://www.unboundmedicine.com/medline/citation/32583809/COVID_19_Associated_Hemophagocytic_Lymphohistiocytosis_and_Coagulopathy:_Targeting_the_Duumvirate_ DB - PRIME DP - Unbound Medicine ER -