Hemophagocytic syndrome secondary to SARS-Cov-2 infection: a case report

Abstract

Background Hemophagocytic syndrome (HPS) is a severe hyperinflammatory disease, whose diagnosis is based on the HLH-2004 criteria. In secondary forms of HLH (sHLH), the primary goal is treating the triggering factors such as COVID-19 (Coronavirus disease 2019). The link between the cytokine storm related to COVID-19 and development of sHLH has already been reported since the onset of pandemic, but little is known about clinical manifestations of HLH which develop after the patient’s recovery from mild symptomatic or asymptomatic Sars-CoV-2 infection. Case presentation We describe the case of a woman diagnosed with sHLH related to previous Sars-CoV-2 infection and successfully treated with steroids, colchicine, etoposide and ruxolitinib. Conclusions Our report suggests that HLH-like syndrome might be secondary to Sars-CoV-2 infection, even if the patient utterly recovered from the mildly symptomatic viral infection. In addition, we underline the treatment with low dose ruxolitinib plus etoposide as a potential choice for Sars-CoV-2 infection related HLH.

Hemophagocytic Lymphohistiocytosis Caused by a Severe Epstein-Barr Virus Infection in a Young Patient Presenting With Hiccups

“…Infections are the most common cause of HLH with EBV being the most common infectious trigger of HLH [2]. Other viruses implicated in HLH include cytomegalovirus, parvovirus, herpes simplex virus, varicella-zoster virus, measles virus, human herpes virus 8, influenza virus, and SARS-CoV-2 [6,14]. Although HLH has been found in association with Xlinked lymphoproliferative disease following an EBV infection, the majority of EBV-induced HLH cases occur in previously healthy and immunocompetent children similar to our patient [15].…”

Ruxolitinib as an Effective Treatment for Hemophagocytic Lymphohistiocytosis Secondary to SARS-Cov-2 Infection: A Case Report

“…The patient was living in the most affected area by COVID-19 and SARS-Cov-2 immunoglobulins G was found to be positive. 6 The decision to start HLH-directed treatment depends on clinical judgment and assessment of organ function. In addition to managing the triggered factors, standard therapy involves corticosteroids, typically dexamethasone, cyclosporine A, intrathecal therapy, and etoposide, to suppress excess cytokine production by activated T-cells.…”

“…HLH can not only be triggered by severe current SARS-CoV-2 infection, but also occur in patients during recovery from mild symptomatic or asymptomatic Sars-CoV-2 infection. 6 For the patients developing sHLH related to previous SARS-CoV-2 infection, the symptoms may reappear after a symptom-free period and deteriorate rapidly. 5 Similar to our patient, there was a previous reported secondary HLH of SARS-Cov-2 infection with negative results for multiple SARS-Cov-2 nasopharyngeal swabs at the time of CSS.…”

“…The treatment with low dose ruxolitinib plus etoposide is a potential choice for COVID-19-related HLH. 6 Here, we present a case of HLH secondary to SARS-Cov-2 infection, which had rapid response to ruxolitinib.…”

Hémophagocytose lymphohistiocytaire réfractaire dans le cadre d’une infection pulmonaire à SARS CoV 2 en réanimation

“…Le chevauchement des caractéristiques cliniques et biologiques entre une infection sévère à SARS CoV-2 et l’HLH rendent le diagnostic difficile en réanimation [1] . Strauss et al rapportent sur une série autopsique de patients décédés en service de soins intensifs des images d’hémophagocytose sur les prélèvements anatomopathologiques dans 64 % des cas sans critère biologique de HLH associé [2] . Cet exemple illustre que le diagnostic doit reposer sur un faisceau d’arguments, pouvant s’aider du HScore et des critères HLH-2004.…”