COVID-19 Associated Hemophagocytic Lymphohistiocytosis and Coagulopathy: Targeting the Duumvirate
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COVID-19 Associated Hemophagocytic Lymphohistiocytosis and Coagulopathy: Targeting the Duumvirate SUKRITA BHATTACHARJEE1, MAINAK BANERJEE2 AND RIMESH PAL3
From 1Institute of Hematology and Transfusion Medicine, Medical College and Hospital, and 2Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India; and 3Post Graduate Institute of Medical Education and Research, Chandigarh, India. Correspondence to: Dr Sukrita Bhattacharjee, Department of Hematology, Institute of Hematology and Transfusion Medicine, Medical College and Hospital, Kolkata, India. [email protected] 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.
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-CoV2”, “HLH”, “HScore”, “coagulopathy”, “D-dimer”, “cytokine storm”, “children” and “pediatrics” with interposition of Boolean operator “AND”.
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.
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
Keywords: Cytokine storm, Disseminated intravascular coagulation, Immunosuppression, Management, SARS-CoV-2. Published online: June 24, 2020; PII: S097475591600204
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ovel coronavirus disease 2019 (COVID-19) has emerged as a pandemic, claiming over 350,000 lives worldwide. Disease mortality has been mostly attributed to viral pneumonia, complicated by acute respiratory distress syndrome (ARDS) and/or sepsis. A cytokine storm like picture in peripheral blood, as evident by significantly higher plasma levels of interleukin (IL)-2, IL-7, tumor necrosis factor-α (TNF-α), granulocyte colonystimulating factor (GCSF)
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