etoposide + cyclosporine + dexamethasone + allogeneic hematopoietic cell transplantation

This therapeutic regimen is a risk-stratified, modified version of the HLH-2004 protocol designed specifically for adult patients with secondary hemophagocytic lymphohistiocytosis (HLH). Developed by the Asan Medical Center, the protocol utilizes a combination of the cytotoxic agent etoposide and immunosuppressive therapy (IST) consisting of cyclosporine and dexamethasone. Patients are stratified by serum ferritin levels (mild, moderate, severe) to determine the intensity of the initial therapy. For patients with refractory or reactivated disease, the protocol incorporates allogeneic hematopoietic cell transplantation (alloHCT) as a definitive curative intent therapy. The regimen aims to suppress the hyperinflammatory cytokine storm characteristic of HLH while minimizing the toxicities often associated with pediatric-focused protocols when applied to adult populations.

Other names

Modified HLH-2004 ProtocolIST and/or alloHCTimmunosuppressive therapy + allogeneic hematopoietic cell transplantation-Asan Medical Center-hemophagocytic lymphohistiocytosis