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Prevalence and Outcome of Secondary Hemophagocytic Lymphohistiocytosis Among SIRS Patients: Results from a Prospective Cohort Study

1
Division of Infectious Diseases and Tropical Medicine, Department of Medicine I, Medical University of Vienna, 1090 Vienna, Austria
2
Division of Nephrology and Dialysis, Department of Medicine III, Medical University of Vienna, 1090 Vienna, Austria
3
Division of Bone Marrow Transplantation, Department of Medicine I, Medical University of Vienna, 1090 Vienna, Austria
4
Department of Laboratory Medicine, Medical University of Vienna, 1090 Vienna, Austria
*
Author to whom correspondence should be addressed.
J. Clin. Med. 2019, 8(4), 541; https://doi.org/10.3390/jcm8040541
Received: 7 March 2019 / Revised: 12 April 2019 / Accepted: 16 April 2019 / Published: 19 April 2019
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Abstract

Secondary hemophagocytic lymphohistiocytosis (sHLH) is a life-threatening condition clinically presenting as SIRS (Systemic Inflammatory Response Syndrome). However, there is no comprehensive data concerning diagnostic algorithms, prevalence, outcome and biomarker performance in SIRS patients. We conducted a prospective observational cohort study on 451 consecutive patients fulfilling ≥2 SIRS criteria. The Hscore and the HLH-2004 criteria were used to determine the presence of sHLH, and the correlation of the screening-biomarkers ferritin, sCD25, and sCD163 with both scores was assessed. Out of 451 standard-care SIRS patients, five patients had high Hscores (≥169), suggesting incipient or HLH-like disease, and these patients were in urgent need for intensified therapy. However, none of these patients fulfilled five HLH-2004 criteria required for formal diagnosis. From the studied biomarkers, ferritin correlated strongest to both the HLH-2004 criteria and the Hscore (rs = 0.72, 0.41, respectively), and was the best predictor of 30-day survival (HR:1.012 per 100 μg/L, 95% CI: 1.004–1.021), when adjusted for patient’s age, sex, bacteremia and malignant underlying-disease. Also, the HLH-2004 (HR per point increase: 1.435, 95% CI: 1.1012–2.086) and the Hscore (HR per point increase:1.011, 95% CI: 1.002–1.020) were independent predictors of 30-day-survival. The Hscore detected patients in hyperinflammatory states requiring urgent therapy escalation. Degrees of hyperinflammation, as assessed by ferritin and both HLH scores, are associated with worse outcomes. View Full-Text
Keywords: hemophagocytic lymphohistiocytosis; HLH; hemophagocytosis; SIRS; survival; outcome; prevalence hemophagocytic lymphohistiocytosis; HLH; hemophagocytosis; SIRS; survival; outcome; prevalence
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Gualdoni, G.A.; Hofmann, G.A.; Wohlfarth, P.; Winkler, H.-M.; Winkler, S.; Haslacher, H.; Thalhammer, R.; Makristathis, A.; Ratzinger, F.; Burgmann, H. Prevalence and Outcome of Secondary Hemophagocytic Lymphohistiocytosis Among SIRS Patients: Results from a Prospective Cohort Study. J. Clin. Med. 2019, 8, 541.

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