Hemophagocytic lymphohistiocytosis in a patient with COVID-19 treated with tocilizumab: a case report

Abstract

Background The understanding of coronavirus disease 2019 (COVID-19) is rapidly evolving. Although it is primarily a respiratory illness, other manifestations, such as Guillain-Barré syndrome, immune thrombocytopenia, and immune-mediated thrombotic thrombocytopenic purpura, have been described. We present a case of a patient with hemophagocytic lymphohistiocytosis secondary to COVID-19 treated with tocilizumab with a marked biochemical improvement. Case presentation In this case report we present a Caucasian patient with COVID-19 who developed a marked elevation of inflammatory parameters with ferritin 36,023 μg/L, but also elevated C-reactive protein 334 mg/L and lactate dehydrogenase 1074 U/L, 1 week after admission to the intensive care unit. He met five of eight criteria for hemophagocytic lymphohistiocytosis, but he lacked the high fever and cytopenia seen in the majority of cases. He was treated with tocilizumab, a monoclonal antibody targeting the interleukin-6 receptor, and over the next days, a rapid decrease in ferritin and C-reactive protein levels was observed. However, his respiratory failure only improved gradually, and he was weaned off the respirator 11 days later. Conclusion COVID-19 may induce a hyperinflammatory clinical picture and in some cases develop into hemophagocytic lymphohistiocytosis. In our patient’s case, therapeutic interleukin-6 blockade abrogated signs of hyperinflammation but did not seem to improve pulmonary function. Measurement of ferritin and C-reactive protein, as well as quantification of interleukin-6 on indication, should be performed in patients with severe COVID-19. Specific treatment in such patients must also be contemplated, preferably in randomized controlled trials.

Secondary Hemophagocytic Lymphohistiocytosis in Post-COVID-19 Patients: A Report of Two Cases

“…HLH in COVID-19 positive patients is reported in various case reports [ 14 , 15 ]. However, the cases of sHLH in patients who have recovered from COVID-19 and are RAT and RT-PCR negative are sparse.…”

Complicated case of COVID-19 disease with overlapping features of thrombotic thrombocytopenic purpura and haemophagocytic lymphohistiocytosis

“…It is well known that both primary and secondary HLH can be triggered by many viral infections like the Epstein-Barr virus and others. 6 The mechanism behind SARS-CoV-2 and the 7 The mechanism of TTP in COVID-19 disease is even poorly understood. Altowyan E et al have reported a 39-year-old man, with COVID-19 disease and TTP, which was suspected by peripheral smear examination showing evidence of thrombocytopenia and microangiopathic haemolytic anaemia.…”

“…The patient was treated with tocilizumab (anti-IL-6 antibody) with other supportive care and his inflammatory parameter improved gradually. 7 …”

A Comparison of the Clinical, Viral, Pathologic, and Immunologic Features of Severe Acute Respiratory Syndrome (SARS), Middle East Respiratory Syndrome (MERS), and Coronavirus 2019 (COVID-19) Diseases

“…87 However, so far morphological evidence of prominent erythrophagocytosis has been found in only a few cases. 88 Lymphoid depletion in lymph nodes and spleen has been reported with a reduction of white pulp with loss of peripheral cuff lymphocytes. 12 This is consistent with a viral attack on immunocytes and the lymphopenia that may be seen at the time of presentation.…”