Could hemophagocytic lymphohistiocytosis be the core issue of severe COVID-19 cases?

BMC Med. 2020 Jul 15;18(1):214. doi: 10.1186/s12916-020-01682-y.

Abstract

Background: COVID-19, a disease caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), commonly presents as fever, cough, dyspnea, and myalgia or fatigue. Although the majority of patients with COVID-19 have mild symptoms, some are more prone to serious outcomes, including pneumonia, acute respiratory distress syndrome (ARDS), and even death. Hemophagocytic lymphohistiocytosis (HLH) is a severe, life-threatening inflammatory syndrome associated with intense cytokine release (also known as a "cytokine storm"). Similar to COVID-19, HLH is characterized by aggressive course leading to multi-organ failure.

Main text: The purpose of this review article is to draw attention to the possibility of the complication of HLH in patients with the severe course of COVID-19. Indeed, some of the clinical characteristics observed in the more severe cases of COVID-19 are reminiscent of secondary HLH (which can be triggered by infections, malignancies, rheumatological diseases, or autoimmune/immunodeficiency conditions). The pathogenesis of SARS-CoV-2 infection also suggests that HLH or a similar hyperinflammatory syndrome is the cause of the severe course of the infection.

Conclusion: The pathogenesis and clinical symptoms of severe COVID-19 indicate that an increased inflammatory response corresponding to HLH is occurring. Therefore, patients with severe COVID-19 should be screened for hyperinflammation using standard laboratory tests to identify those for whom immunosuppressive therapy may improve outcomes.

Keywords: Coronavirus; Coronavirus disease 2019; Hemophagocytic lymphohistiocytosis.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Betacoronavirus
  • COVID-19
  • Coronavirus Infections / complications*
  • Coronavirus Infections / therapy
  • Cytokine Release Syndrome / virology*
  • Humans
  • Lymphohistiocytosis, Hemophagocytic / diagnosis
  • Lymphohistiocytosis, Hemophagocytic / therapy
  • Lymphohistiocytosis, Hemophagocytic / virology*
  • Pandemics
  • Pneumonia, Viral / complications*
  • Pneumonia, Viral / therapy
  • SARS-CoV-2