Abstract
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome. Complement component 3 (C3), a central effector molecule in three separate complement pathways, has been linked to inflammatory diseases. Hence, we aimed to investigate the clinical significance of C3 in adult HLH. In this study, our retrospective cohort involved patients meeting ≥5 of 8 HLH-2004 criteria were classified as the HLH group (n=627), while those meeting <5 criteria were the partial HLH group (n=588). C3 was significantly lower in the HLH group compared to the partial HLH group (P<0.0001) and low C3 was an independent factor predicting progression from partial HLH to HLH (OR=3.94, P<0.001). Low C3 was associated with more severe cytopenia, coagulation abnormalities, and liver dysfunction. Additionally, patients with low C3 had poorer overall survival (P=0.00099) and low C3 was an independent risk factor for early death in HLH (HR=1.64, P=0.019). The majority of HLH patients had normal C3 before HLH onset, followed by a decline after HLH development (P<0.0001). Moreover, survivors showed increasing C3 (P=0.0003), while non-survivors exhibited decreasing C3 (P=0.90). In conclusion, our study identified C3 as a valuable predictive and prognostic biomarker in adult HLH. Monitoring the dynamic changes in C3 levels may reflect therapeutic response and timely guide clinical interventions.
Published Version
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