Table 3.
Consensus recommendations on risk stratification and management of HLH.
| S. No. | Recommendation | Grade of evidence | Level of agreement (%) |
|---|---|---|---|
| 10. | Therapy should be risk-stratified according to disease severity. High-intensity, HLH-directed regimens are indicated for life-threatening hyperinflammation; less intensive approaches are appropriate for milder presentations, balancing therapeutic benefit against treatment-related toxicity. | Moderate | 100% (15/15) |
| 11. | Patients with suspected or confirmed HLH should receive immediate supportive care, including hemodynamic stabilization, management of coagulopathy/bleeding, infection coverage, and organ support, as needed. | Moderate | 100% (15/15) |
| Primary/familial HLH | |||
| 12. | For primary/familial HLH, HLH-directed therapy, including dexamethasone and etoposide, with or without CNS-directed therapy, should be initiated promptly. | Moderate | 100% (15/15) |
| 13. | In patients who respond to induction therapy, treatment should be continued with close monitoring of clinical and laboratory parameters, i.e., fever, ferritin, CBC, fibrinogen, liver function, and CNS status. | Low | 100% (15/15) |
| Secondary HLH | |||
| 14. | Concomitant management of the specific underlying trigger alongside HLH-directed therapy improves outcomes and avoids unnecessary immunosuppression when a reversible trigger is present. | Low | 100% (15/15) |
| 15. | In severe or progressive disease, treatment should include prompt initiation of corticosteroids and management of the underlying trigger, with consideration of etoposide in severe cases. If etoposide is used, the dose and frequency should be adjusted to 50–100 mg/m² (from 150 mg/m²) and limited to 1–2 doses in older patients. | Moderate | 93% (13/14)* |
| 16. | In milder or stable cases, initial management may focus on treating the underlying trigger, with very close monitoring and rapid escalation if there is no clinical response. | Low | 93% (14/15) |
*Abstention was recorded for this statement, as the expert indicated that it was outside their scope of practice.
Level of agreement was calculated as the proportion of respondents selecting either “agree” or “somewhat agree.”
CBC, complete blood count; CNS, central nervous system; HLH, hemophagocytic lymphohistiocytosis.