While in genetic HLH an intensive immune-chemotherapy protocol (HLH – 2004 protocol) could reduce the mortality significantly, there are no standardized treatment regimens for acquired HLH. However, this is difficult since the underlying diseases and triggers in adults are very different and an individual therapy is required.
On the one hand, in patients with a fulminant course of (EBV-)HLH there is a significant improvement of survival by rapidly initiated immune-chemotherapy including etoposide (especially in severe EBV – HLH , Imashuku et al 2001), on the other hand, there is the risk of overtreatment and unnecessary immunosuppression in unclear cases.
The therapy in adult HLH patients should be individualized because of the heterogeneity of the underlying disease and triggers. In mild cases a “watch and wait – strategy” is possible or a short-term course of dexamethasone and immunoglobulins can be used.
In any case there should be an intensive search for and treatment of possible infectious HLH-triggers.