Secondary Hemophagocytic Syndrome in an 82-Year-Old Covid-19 Patient
Francesk Mulita1, Nikoleta Oikonomou2, Levan Tchabashvili1, Elias Liolis3 and Ioannis Maroulis1
1Department of General Surgery, General University Hospital of Patras, Achaia, Greece
2Department of Pediatrics, Neonatal Intensive Care Unit, General University Hospital of Patras, Achaia, Greece
3Department of Internal Medicine, General University Hospital of Patras, Achaia, Greece
Received Date: 20/07/2020; Published Date: 30/07/2020
*Corresponding author: Francesk Mulita, Department of General Surgery, General University Hospital of Patras, Mezonos 2, 26223, Patras, Greece
Cite this article: Francesk Mulita, Secondary Hemophagocytic Syndrome in an 82-Year-Old Covid-19 Patient. Op Acc J Bio Sci & Res 3(5)- 2020.
Background: Postoperative fever is defined as a temperature greater than 38oC on two consecutive postoperative days or greater than 39oC on any postoperative day. Case presentation: We herein report a case of an 82-year-old male who presented to our hospital complaining of abdominal pain and fever. Chest radiography of this patient showed right pleural thickening, but his first Covid-19 test was negative. After 7 days of hospitalization, his general situation became worse as well as he became hypoxic to 80% oxygen saturation and remained spiking fevers up to 39oC. He was positive to the second Covid-19 test. The patient was intubated. According to blood results tests our patient had secondary hemophagocytic syndrome, which is a life-threatening syndrome of excessive immune activation that can also be induced by SARS-CoV-2 infection. Unfortunately, he died and there was no further management. Conclusions: COVID-19 was declared as a global pandemic. It is very important nowadays this pathology to be excluded from all patient presenting to emergency departments complaining of fever.
Keywords: COVID-19; Secondary hemophagocytic syndrome; Postoperative fever
Postoperative fever is defined as a temperature greater than 38 °C on two consecutive postoperative days or greater than 39 °C on any postoperative day . We herein report a case of an 82-year-old male who presented to our Emergency Department complaining of abdominal pain and fever.
An 82-year-old male with free medical history presented to our hospital with fever and abdominal pain for 3 days which was not associated with heartburn, vomiting, melena or haematemesis. He had undergone open cholecystectomy one year ago and an open postoperative hernia repair in the right Kocher’s incision was performed 25 days ago. Patient’s temperature was 38.4 °C, heart rate was 107 beats per minute, blood pressure was 161/113 and respiratory rate was 19 breaths per minute. On examination, it was revealed moderate tenderness in the region of upper abdomen with no rebound tenderness. Bowel sounds were audible, and rectal examination was normal. The initial haemoglobin was 11.2 g/dL, white blood cells were 5.11 K/μl, platelets were 210.000 K/μl and C-reactive protein level was 10 mg/dL. Liver and renal function test and serum amylase were normal. Chest radiography showed right pleural thickening (Figure 1). A further computed tomography (CT) scan of the abdomen and chest demonstrated pleural thickening of the inferior half of the right middle lobe (Figure 2) as well as low volume of free intra-peritoneal fluid. Because of the history of recent surgery the patient was initially hospitalized in the surgical clinic, as his first Covid-19 test was negative. The patient remained in the surgical clinic for 4 days without having any surgical problem and received intravenous antibiotics empirically. During his hospitalization he had no abdominal pain and was started on oral diet. However, his chest radiography showed no improvement (Figure 3) and he was referred to internal medicine department for further management.
Figure 1: Chest radiography showing right pleural thickening.
Figure 2: Chest CT scan demonstrating pleural thickening of the inferior half of the right middle fissure with adjacent subsegmental atelectasis of the lateral segment of the right middle lobe.
Figure 3: Chest radiography showing right pleural thickening after 4 days of hospitalization.
A second Covid-19 test was sent while the patient was hospitalized in internal medicine clinic and it was positive. After 4 days of hospitalization there, his general situation became worse as well as he became hypoxic to 80% oxygen saturation while on nasal cannula and remained spiking fevers up to 39oC. He was intubated and transferred to the intensive care unit (ICU) for further management and was switched to ceftriaxone 1 g intravenously daily and azithromycin 500 mg via orogastric tube daily and was started on hydroxychloroquine 400 mg loading dose followed by 200 mg twice daily for a 7-day course. Seven days after intubation his haemoglobin was 10.5 g/dL, white blood cells were 2.73 K/μl, platelets were 65.000 K/μl and C-reactive protein level was 3.83 mg/dL. Patient’s ferritin was 18.111 ng/ml and his transaminases, LDH and triglycerides were elevated. According to these findings there was a suspicion of hemophagocytic syndrome in our patient. Unfortunately, he died the same day and no further management was made.
Coronavirus disease 2019 (COVID-19) is a disease caused by a novel coronavirus, called severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) . The incubation period for COVID-19 is thought to be approximately 14 days following exposure, with most cases occurring four to five days after exposure . Clinical manifestations of Covid-19 disease show a wide spectrum of severity. Generally, the fatality rate is thought to be approximately 2.3 %. The most common symptoms reported in coronavirus infection are the following: fever, cough, dyspnea, sore throat, rhinitis, fatigue and diarrhea. Most of the deaths reported are due to acute respiratory failure, ARDS and shock and multiorgan failure [4-5].
Diagnosis of the SARS-CoV-2 infection is based on laboratory findings, and specific tests. Most common laboratory findings include: Leukopenia, leukocytosis, lymphopenia and high levels of lactate dehydrogenase and ferritin . Chest CT scanning in patients suffered from SARS-CoV-2 infection usually show ground-glass opacities and infiltrating consolidation [7-8].
The appropriate management and treatment of SARS-CoV-2 infection is under investigation. During the outbreak of the COVID-19 hydroxychloroquine, chloroquine, or lopinavir/ritonavir were more commonly used, but eventually these drugs showed little or no reduction in mortality rates compared with standard of care. No vaccine is currently available for SARS-CoV-2 . Recently, antibodies tests are available detecting specific IgM and IgG antibodies against SARS-CoV-2 . Hemophagocytic lymphohistiocytosis (HLH) is an aggressive and life-threatening syndrome of excessive immune activation. It most commonly appears in infancy, although it has been seen in all age groups . It is categorized into two major types: HLH syndrome – A condition of pathologic immune activation that is often associated with genetic defects of lymphocyte cytotoxicity and HLH disease – A condition associated with infections, malignancy and rheumatologic conditions and is mostly seen in adults. Viral pathogens that are associated with HLH are Epstein-Barr virus (EBV), cytomegalovirus (CMV), parvovirus, herpes simplex virus, varicella-zoster virus, measles virus, human herpes virus 8 and H1N1 influenza virus. It is worth mentioning that HLH is probably associated also with SARS-CoV-2 [12-15].
Clinical manifestations of HLH include: Fever, cytopenia (especially anemia and thrombocytopenia), splenomegaly, dyspnea, ARDS syndrome, renal failure, severe hypotension and skin manifestations such as generalized rashes, erythroderma, edema, petechiae, and purpura . Diagnosis is based on laboratory findings and bone marrow biopsy. Characteristic laboratory findings include: cytopenias in the peripheral blood, hypertriglyceridemia, hypofibrinogenemia, high ferritin levels (>500 mcg/L) and elevated levels of LDH, hemophagocytosis, low/absent NK cell activity and soluble CD25 elevation. Bone marrow evaluation is recommended for the diagnosis of HLH. Infiltration of the bone marrow by activated macrophages is commonly seen in patients with HLH and supports the diagnosis . Puja Mehta and colleagues suggest using the HScore to detect hyperinflammatory states in patients with coronavirus disease 2019 (COVID-19). This score validated for the diagnosis of secondary haemophagocytic lymphohistiocytosis (sHLH), a condition that shares a similar cytokine profile to severe COVID-19 .
COVID-19 was declared as a global pandemic. It is very important nowadays this pathology to be excluded from all patient presenting to emergency departments complaining of fever. Secondary hemophagocytic syndrome is life-threatening syndrome of excessive immune activation that can also be induced by SARS-CoV-2 infection.
Declaration of Conflicting Interests
The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
The authors received no financial support for the research, authorship, and/or publication of this article.
Written informed consent was obtained from the patient for their anonymized information to be published in this article and approved by local ethics committee.
FM, NO, LT and EL: contributed to the clinical data collection and prepared the case report. FM and IM: contributed to the design of the case report presentation and performed the final revision of the manuscript.
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