The classical HLH-94 protocol includes:

  • Corticosteroids – usually dexamethasone in primary forms and methylprednisolone in secondary forms
  • Etoposide – a chemotherapy agent with high specificity against T-cells
  • Cyclosporin A
  • Methotrexate

    Other drugs that are commonly used include

  • Anakinra (an interleukin-1 inhibitor)
  • Immunoglobulin
  • Alemtuzumab
  • Tocilizumab
  • The heterogeneity of adult HLH prohibits a “1-size-fits-all protocol”
  • Management is highly specialised and requires expert input
  • A summary of commonly used management options is provided in the American Society of Haematology Guidance