Table 4.
Limitations of conventional therapy in HLH.
| S. No. | Recommendation | Grade of evidence | Level of agreement (%) |
|---|---|---|---|
| 17. | Conventional immunochemotherapy (e.g., corticosteroids plus etoposide) is constrained by significant toxicity and limited durable efficacy in adults and complex HLH cases, supporting the need for risk-adapted and alternative therapeutic strategies beyond etoposide-based regimens. | Moderate | 100% (15/15) |
| 18. | Standard HLH therapy may be impractical or unsafe in patients with severe organ dysfunction, comorbidities, or frailty, necessitating alternative or tailored approaches. | Moderate | 100% (15/15) |
| 19. | As conventional therapy does not directly address persistent triggers, it may be insufficient in cases driven by chronic infection, malignancy, or complex immune dysregulation. | Low | 93% (14/15) |
| 20. | There is a need for targeted, risk-adapted therapies to overcome the toxicity, feasibility, and efficacy limitations of conventional treatment in HLH. | Low | 100% (15/15) |
Level of agreement was calculated as the proportion of respondents selecting either “agree” or “somewhat agree”.
HLH, hemophagocytic lymphohistiocytosis.