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Hemophagocytic syndrome: a potential COVID-19 complication

A 62-year-old male smoker who was an alcoholic and had diabetes was being treated for mediastinitis after surgical myocardial revascularization when he presented with worsening of the respiratory pattern despite overall improvement, requiring endotracheal intubation. The patient underwent a CT scan of the chest that showed findings consistent with COVID-19 (Figures 1A and 1B), which was later confirmed by RT-PCR. Seventeen days after the diagnosis of COVID-19, the patient presented with pancytopenia and increased levels of ferritin and IL-2 receptor. Hemophagocytic syndrome (HPS) was therefore suspected. A myelogram confirmed the diagnosis of HPS. Treatment consisted of i.v. immunoglobulin for two days. Thirteen days after the diagnosis of HPS, the patient experienced decreased oxygen saturation and tachypnea. A new CT scan of the chest showed new cavitating pulmonary lesions, superimposed on the pulmonary findings of COVID-19 (Figures 1C and 1D). Bronchoscopy revealed grossly necrotic tissue and bronchial infection with Aspergillus fumigatus (Figure 1E). The patient was started on anidulafungin in an attempt to contain the fungal infection; however, given the severity of his condition, he died three days after treatment initiation.

Figure 1
In A and B, respectively, axial and coronal CT scans of the chest showing extensive bilateral ground-glass opacities, associated with fine reticulation and small foci of consolidation, consistent with COVID-19. Note also bilateral pleural effusions. In C and D, respectively, sagittal and axial CT images of the chest showing cavitated pulmonary lesions (arrows) in the left upper lobe, superimposed on the findings of COVID-19. In E, bronchoscopic findings of grossly necrotic tissue and yellowish secretion, suggesting fungal colonization.

HPS is a rare and severe complication characterized by fever, hepatosplenomegaly, cytopenia, and activated macrophages in hematopoietic organs. It is often related to infectious diseases, and it can increase the risk of developing opportunistic infections.11 Balci YI, Özgürler Akpinar F, Polat A, Kenar F, Tesi B, Greenwood T, et al. A Hemophagocytic Lymphohistiocytosis Case with Newly Defined UNC13D (c.175G>C; p.Ala59Pro) Mutation and a Rare Complication. Turk J Haematol. 2015;32(4):355-358. https://doi.org/10.4274/tjh.2014.0416
https://doi.org/10.4274/tjh.2014.0416...
New evidence indicates that HPS can occur in the context of COVID-19,22 Moore JB, June CH. Cytokine release syndrome in severe COVID-19. Science. 2020;368(6490):473-474. https://doi.org/10.1126/science.abb8925
https://doi.org/10.1126/science.abb8925...
,33 Faguer S, Del Bello A, Abravanel F, Nicolau-Travers ML, Kamar N. Tocilizumab for Hemophagocytic Syndrome in a Kidney Transplant Recipient With COVID-19 [published online ahead of print, 2020 May 18]. Ann Intern Med. 2020;10.7326/L20-0419. https://doi.org/10.7326/L20-0419
https://doi.org/10.7326/L20-0419...
carrying an ominous prognosis.

REFERENCES

  • 1
    Balci YI, Özgürler Akpinar F, Polat A, Kenar F, Tesi B, Greenwood T, et al. A Hemophagocytic Lymphohistiocytosis Case with Newly Defined UNC13D (c.175G>C; p.Ala59Pro) Mutation and a Rare Complication. Turk J Haematol. 2015;32(4):355-358. https://doi.org/10.4274/tjh.2014.0416
    » https://doi.org/10.4274/tjh.2014.0416
  • 2
    Moore JB, June CH. Cytokine release syndrome in severe COVID-19. Science. 2020;368(6490):473-474. https://doi.org/10.1126/science.abb8925
    » https://doi.org/10.1126/science.abb8925
  • 3
    Faguer S, Del Bello A, Abravanel F, Nicolau-Travers ML, Kamar N. Tocilizumab for Hemophagocytic Syndrome in a Kidney Transplant Recipient With COVID-19 [published online ahead of print, 2020 May 18]. Ann Intern Med. 2020;10.7326/L20-0419. https://doi.org/10.7326/L20-0419
    » https://doi.org/10.7326/L20-0419

Publication Dates

  • Publication in this collection
    21 Sept 2020
  • Date of issue
    2020
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