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. 2026 Sep 14;17:1933805. doi: 10.3389/fimmu.2026.1933805

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.