Hemophagocytic Lymphohistiocytosis in a Patient With COVID-19 Treated With Tocilizumab

A Case Report

Birgitte Tholin; Marit Teigen Hauge; Pål Aukrust; Lutz Fehrle; Tor Henrik Tvedt

Disclosures

J Med Case Reports. 2020;14(187) 

In This Article

Abstract and Introduction

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.

Introduction

Severe coronavirus disease 2019 (COVID-19) infection, particularly in those with acute respiratory distress syndrome (ARDS), is characterized by marked inflammation and immune activation with elevated levels of inflammatory cytokines, such as interleukin (IL)-6 and tumor necrosis factor.[1] Significantly elevated levels of ferritin as a marker of extensive macrophage activation have also been reported.[2] We describe a case of a patient with a marked systemic immune reaction with features of hemophagocytic lymphohistiocytosis (HLH) who was treated with the IL-6 receptor antagonist tocilizumab.

processing....