Low-dose Emapalumab Combined with Chemotherapy for Adult Patients with Epstein-Barr Virus-associated Hemophagocytic Lymphohistiocytosis

Hemophagocytic lymphohistiocytosis (HLH) is a severe disorder with poor clinical outcomes. Use of emapalumab, an IFN-γ inhibitor, enables primary HLH control in over 85 % of affected children. However, data on emapalumab use for Epstein-Barr virus-associated HLH (EBV-HLH) are limited. This report presents the cases of three patients with EBV-HLH, highlighting the successful integration of low-dose emapalumab in combination with chemotherapy as a novel therapeutic approach for patients diagnosed with EBV-HLH. This regimen resulted in rapid disease symptom control and hematological parameter improvement and facilitated successful stem cell transplantation. This report highlights the potential of low-dose emapalumab combined with chemotherapy as an effective bridging therapy to allogenic hematopoietic stem cell transplantation in EBV-HLH patients.