Hemoadsorption Treatment with CytoSorb® in Probable Hemophagocytic Lymphohistiocytosis: A Role as Adjunctive Therapy?

Abstract

Acute hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease, with an annual incidence of 1 : 800,000 people. The disease is characterized by a cytokine storm, with concomitant macrophage and natural killer (NK) cell activation; death can occur from multiple organ failure or complications such as bleeding diathesis. Therefore, HLH treatment remains a challenging one. We hereby present a case of a 76-year-old man with severe HLH in whom hemoadsorption was successfully applied. Due to the failure of the immunomodulatory therapy , continuous venovenous hemodiafiltration therapy with the CytoSorb® adsorber was successfully applied for 48 hours. Upon therapy discontinuation, the biological and clinical condition reverted, unfortunately evolving towards the patient's death.

Figure 1

H-score calculator (H-score calculator redraw ([13])): the probability of HLH increases with the increase of score. The best cutoff value was 169, which corresponds to an accuracy of 90% (sensitivity of 93%, specificity of 86%). In this case report, patient presented a H-index of 229 points (96–99% of probability).

Figure 2

Temporal evolution of clinical and biological data: temporal evolution of IFN-gamma, interleukin (IL) 6 and IL8 cytokines' serum values, biological data like ferritin, C-reactive protein (CRP), and clinical data such as Glasgow Coma Scale (GCS). Days refer to the day of ICU treatment. Shaded blue areas are the days of CytoSorb® adsorbent application through CVVHDF.