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
