Hemophagocytic Lymphohistiocytosis in a Patient With Acute Respiratory Distress Syndrome Secondary to SARS-CoV-2 Infection

Graziana Francesca Greco, Fabio Spreafico, Domenica Di Costanzo, Antonietta Pecoriello, Martina Garuti, Francesco Inglese, Giulia Cervi, Giuseppe De donno, Massimiliano Beccaria


Hemophagocytic lymphohistiocytosis (HLH) is a potentially life-threatening entity resulting from the dysregulated activation of T-lymphocytes. Secondary HLH (sHLH) complicates various medical conditions. Similar to 2009 H1N1 influenza, severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection appears to trigger a cytokine storm in a subset of individuals. The patient of 64 years old presented himself in the emergency department with cough and fever, and was subjected to the nasopharyngeal swab and tested positive for SARS-CoV-2, as expected from epidemiological data. Hospitalized in cohort isolation, he initially presented a multi-organ impairment and specifically a worsening of his renal function, initial coagulopathy, lymphopenia modest thrombocytopenia and systemic inflammatory commitment. During the course of the 6th day, a picture of a probable cytokine storm and clear multiple organ failure (MOF) was evident. Therefore a clinical diagnosis was made according to HScore criteria of secondary hemophagocytic lymphohistiocytosis. We started high-dose steroid therapy (dexamethasone 8 mg bid). And after 36 h, the patient was significantly improved: alert, oriented, weaned from non-invasive ventilation (NIV) and stable blood chemistry. In conclusion, in coronavirus disease 2019 (COVID-19) positive patients with evidence of cytokine storm and acute respiratory distress syndrome (ARDS), the levels of blood triglycerides and ferritin are useful and often decisive to comfort the diagnosis of sHLH if cytopenia of one or more lines is revealed.

J Med Cases. 2020;11(10):327-329
doi: https://doi.org/10.14740/jmc3515


Hemophagocytic lymphohistiocytosis; SARS-CoV-2; ARDS

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