What is the HLH-94 treatment protocol?

By William Aird

Etoposide- and dexamethasone-based treatment protocol for patients with HLH:

  • Standard of care in children.
  • A more individualized approach regarding dosing (for example IV dexamethasone 10 mg/m2/day with or without modified dose of etoposide) and duration of HLH-94 is usually adopted in adults.

Consists of:

  • Corticosteroids
  • Cyclosporine A (CSA)
  • Intrathecal therapy
  • Etoposide

Goals are to:

  • Delete activated T cells.
  • Suppress inflammatory cytokine production.
Figure 1. Overview of the HLH-94 treatment protocol. Note that dose and frequency adjustments of this protocol are advised for adult patients. Source.