In the differential diagnosis of sepsis and hemophagocytic lymphohistiocytosis, procalcitonin and C-reactive protein (CRP) may be as determinant as ferritin

Abstract

In the differential diagnosis of sepsis and hemophagocytic lymphohistiocytosis, procalcitonin and C-reactive protein (CRP) may be as determinant as ferritin

Abstract

Background: Hemophagocytic lymphohistiocytosis (HLH) and sepsis frequently appear as overlapping diagnoses in intensive care units. It is necessary to distinguish HLH, which has a very high mortality, from sepsis. In this study, we wanted to draw attention to the potential of procalcitonin (PCT) and C-Reactive Protein (CRP) as a marker like ferritin in differential diagnosis. Thus, HLH can be diagnosed as early as possible and the necessary aggressive immunosuppressive therapy can be added to the existing treatment. Methods: All of the patients in the sepsis clinic who meet the HLH criteria Group HLH; patients not meeting the HLH criteria were defined as Group non-HLH. Files of all patients were reviewed in regard to HLH diagnosis criteria and H score. Results: There were 16 patients in Group HLH and 15 in Group non-HLH. CRP and PCT levels were significantly lower (p: 0.007 and p<0.001, respectively) and ferritin levels were higher (p<0.001) in Group HLH. Hyperferritinemia was present in 15 (94%) and hemophagocytosis in bone marrow in 10 (63%) patients. In group HLH patients, the H score was 258 (230.7-281.5). Conclusion: In critically ill children with suspected sepsis, if CRP and PCT values- two of acute phase reactants- do not explain the patient's poor clinical condition, HLH, which is an often overlooked diagnosis, must be excluded.

Keywords

• ferritin; Hemophagocytic lymphohistiocytosis
• Children
• procalcitonin; sepsis

References

1. 1.Henter JI, Horne A, Aricó M, et al. HLH‐2004:diagnostic and therapeutic guidelines forhemophagocytic lymphohistiocytosis. Pediatr BloodCancer. 2007;48(2):124-31.
2. 2.Goldstein B, Giroir B, Randolph A. Internationalpediatric sepsis consensus conference: definitionsfor sepsis and organ dysfunction in pediatrics.Pediatr Crit Care Med. 2005;6(1):2-8.
3. 3.Castell JV, Gómez‐lechón MJ, David M, et al. Acute‐phase response of human hepatocytes: regulation ofacute‐phase protein synthesis by interleukin‐6.Hepatology. 1990;12(5):1179-86.
4. 4.Uzzan B, Cohen R, Nicolas P, et al. Procalcitonin asa diagnostic test for sepsis in critically ill adults andafter surgery or trauma: a systematic review andmeta-analysis. Crit Care Med. 2006;34(7):1996-2003.