Many
biomarkers have been proposed for the diagnosis of secondary
hemophagocytic lymphohistiocytosis (HLH) in adults, but comparative studies are lacking. We analyzed
ferritin, glycosylated ferritin, soluble CD25, CD163 and CD14,
IL-6, IFN-γ,
IL-18,
IL-10, IL-1ß, IL-12p70, IL-17α, IP-10, and CXCL9 levels to differentiate HLH from
sepsis in
critically ill patients. Of 120 patients, HLH was confirmed for 14 patients. Among the
biomarkers tested,
ferritin,
IL-18, and
glycosylated ferritin were the most efficient parameters for early diagnosis of HLH. With a sensitivity set at 85%,
ferritin,
IL-18, and
glycosylated ferritin were the
biomarkers with the highest specificity: 84, 79, and 71% respectively. Combining
IL-18 with the HScore provided a new score with an increased specificity compared to the HScore alone, 86% compared to 70% with a sensitivity set at 100%. A distinct
cytokine pattern was highlighted in patients with
malignancy-triggered HLH, with highly increased levels of INF-ɣ and CXCL9, compared to HLH secondary to
infection. This is the largest study available to date, comparing diagnostic
biomarkers for HLH on a cohort of
critically ill adult patients. Serum
ferritin was the most discriminating parameter for early diagnosis of secondary HLH.
IL18*HScore was identified as a highly potential score.