Table 1.
Consensus recommendations for early recognition of HLH.
| S. No. | Recommendation | Grade of evidence | Level of agreement (%) |
|---|---|---|---|
| 1. | All patients with suspected HLH, regardless of age or apparent secondary trigger, should be considered for early comprehensive genetic testing as part of initial evaluation. | Low | 80% (12/15) |
| 2. | Given the very early or sepsis-like presentation of HLH in neonates and infants, clinicians should initiate genetic testing together with HLH-directed investigations at first clinical suspicion rather than sequentially after exclusion of secondary causes. | Moderate | 93% (13/14)* |
| 3. | The presence of persistent fever (>38.5 °C), cytopenias affecting ≥2 of 3 peripheral blood lineages, and elevated ferritin (≥500 ng/mL in children; HScore ferritin bands of 2,000–6,000 ng/mL or >6,000 ng/mL in adults) should trigger immediate HLH assessment using HLH-2004/2024 in pediatrics and HLH-2004/2024 and/or HScore in adults, together with parallel evaluation for underlying triggers and prompt initiation of algorithm-based HLH management without waiting for confirmatory results. | Moderate | 93% (14/15) |
| 4. | In suspected HLH in the GCC, standard workups should include broad viral panels (including HIV, CMV-PCR, EBV-PCR, dengue, influenza, etc.), rather than focusing only on EBV and HIV. | Low | 100% (15/15) |
| 5. | Early organ dysfunction in suspected HLH should prompt immediate referral to centers with ICU and HSCT capability to reduce mortality. | High | 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.”
CMV-PCR, cytomegalovirus polymerase chain reaction; EBV-PCR, Epstein-Barr virus polymerase chain reaction; GCC, Gulf Cooperation Council; HIV, human immunodeficiency virus; HLH, hemophagocytic lymphohistiocytosis; HScore, hemophagocytic lymphohistiocytosis probability score; HSCT, hematopoietic stem cell transplantation; ICU, intensive care unit.