Do you recommend the use of dexamethasone and anakinra as upfront treatment in a pediatric patient with secondary HLH (nonrheumatic) who is too ill to tolerate etoposide?   

If there is no concern for new malignancy or systemic infection, then glucocorticoids are reasonable. I use methylprednisolone up to 30 mg/kg/day (max. one gram). Anakinra is a safe and often effective therapy for secondary HLH. It is quick acting and has a short half-life so I use it early. I gener...

Using Anakinra with dexamethasone is a reasonable approach for a patient with secondary HLH who cannot tolerate etoposide. This treatment regimen is well-documented in the literature. There is a definite trend toward treatment regimens that do not incorporate etoposide, such as emapalumab (currently...