In recent years, there has been a change in the approach to treating patients with hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS) to limit exposure to genotoxic substances like etoposide while tamping down hyperinflammation by focusing on the activity of particular HLH/MAS-associated cytokines. For a study, researchers sought to analyze current initiatives to use cytokine-targeted therapeutics or lower etoposide dosage to treat HLH/MAS.
A growing body of research suggested that cytokine-targeted medications, such as those that block or neutralize the signaling caused by interferon-gamma, interleukin (IL)-1, IL-18, and IL-6, can successfully treat the clinical and laboratory symptoms of HLH/MAS and enhance overall results.
When used to treat HLH/MAS, innovative regimens with lower dosages of etoposide and/or medicines that target cytokines had the potential to reduce side effects from the therapy while also reducing inflammation. It was still unclear whether cytokine-targeted therapies should be used alone or in conjunction with more traditional HLH-directed treatments like dexamethasone and standard-dose or reduced-dose etoposide. Additional research was also needed to better understand which patients are the most suitable candidates to receive cytokine-targeted therapies, to determine the best timing and dose for these treatments, and to determine whether they should be administered alone.
