Table 5.
Consensus recommendations for the treatment of refractory/relapsed/intolerant HLH.
| S. No. | Recommendation | Grade of evidence | Level of agreement (%) |
|---|---|---|---|
| 21. | In GCC patients, genetic predisposition and persistent triggers substantially increase the likelihood of refractory or relapsed HLH, thereby justifying earlier escalation beyond conventional immunochemotherapy. | Low | 100% (15/15) |
| 22. | In GCC patients with refractory, relapsed, or treatment-intolerant HLH, risk-adapted management should incorporate targeted and salvage therapy emapalumab, as part of individualized strategies to achieve disease control and facilitate transition (bridge) to definitive therapies such as HSCT. | Moderate | 100% (15/15) |
Level of agreement was calculated as the proportion of respondents selecting either “agree” or “somewhat agree.”.
GCC, Gulf Cooperation Council; HLH, hemophagocytic lymphohistiocytosis; HSCT, hematopoietic stem cell transplantation.