In the differential diagnosis of sepsis and hemophagocytic lymphohistiocytosis, procalcitonin and C-reactive protein (CRP) may be as determinant as ferritin
In the differential diagnosis of sepsis and hemophagocytic lymphohistiocytosis, procalcitonin and C-reactive protein (CRP) may be as determinant as ferritin
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
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- 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.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.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.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.
- 5.Erenler AK, Yapar D, Terzi Ö. Comparison ofprocalcitonin and c-reactive protein in differentialdiagnosis of sepsis and severe sepsis in emergencydepartment. Dicle Med J. 2017;44(2):175-82.
- 6.Lehmberg K, Pink I, Eulenburg C, et al.Differentiating macrophage activation syndrome insystemic juvenile idiopathic arthritis from otherforms of hemophagocytic lymphohistiocytosis. JPediatr. 2013;162(6):1245-51.
- 7.Kim J, Yoo SW, Kang S-R, et al. Clinical implicationof F-18 FDG PET/CT in patients with secondaryhemophagocytic lymphohistiocytosis. Ann Hematol.2014;93(4):661-7.
- 8.Schram AM, Comstock P, Campo M, et al.Haemophagocytic lymphohistiocytosis in adults: amulticentre case series over 7 years. Br J Haematol.2016;172(3):412-9.
- 9.Thomas D, Shah N, Patel H, et al. Hemophagocyticlymphohistiocytosis: A series of five clinical cases inadult patients at a single institution with a review ofthe literature. N Am J Med Sci. 2015;7(9):415.
- 10.Fardet L, Galicier L, Lambotte O, et al.Development and validation of the HScore, a scorefor the diagnosis of reactive hemophagocyticsyndrome. Arthritis Rheumatol. 2014;66(9):2613-20.
- 11.Machowicz R, Janka G, Wiktor-Jedrzejczak W.Similar but not the same: differential diagnosis ofHLH and sepsis. Crit Rev Oncol Hematol.2017;114:1-12.
- 12.Henter JI. Biology and treatment of familialhemophagocytic lymphohistiocytosis: importanceof perforin in lymphocyte-mediated cytotoxicity and triggering of apoptosis. Med Pediatr Oncol.2002;38(5):305-9.
- 13.Janka G. Familial and acquired hemophagocyticlymphohistiocytosis. Annu Rev Med. 2012;63:233-46.
- 14.Debaugnies F, Mahadeb B, Ferster A, et al.Performances of the H-score for diagnosis ofhemophagocytic lymphohistiocytosis in adult andpediatric patients. Am J Clin Pathol.2016;145(6):862-70.
- 15.Janka G. Hemophagocytic lymphohistiocytosis:when the immune system runs amok. Klin Padiatr.2009;221(05):278-85.
- 16.Castillo L, Carcillo J. Secondary hemophagocyticlymphohistiocytosis and severe sepsis/systemicinflammatory response syndrome/multiorgandysfunction syndrome/macrophage activationsyndrome share common intermediate phenotypeson a spectrum of inflammation. Pediatr Crit CareMed. 2009;10(3):387-92.
- 17.Janka GE. Familial and acquired hemophagocyticlymphohistiocytosis. Eur J Pediatr. 2007;166(2):95-109.
- 18.Allen CE, Yu X, Kozinetz CA, et al. Highly elevatedferritin levels and the diagnosis of hemophagocyticlymphohistiocytosis. Pediatr Blood Cancer.2008;50(6):1227-35.
- 19.Jordan MB, Allen CE, Weitzman S, et al. How I.treat hemophagocytic lymphohistiocytosis. Blood.2011;118:4041-52.
- 20.Lin TF, Ferlic Stark LL, Allen CE, et al. Rate ofdecline of ferritin in patients with hemophagocyticlymphohistiocytosis as a prognostic variable formortality. Pediatr Blood Cancer. 2011;56(1):154-5.
- 21.Bennett TD, Hayward KN, Farris RW, et al. Veryhigh serum ferritin levels are associated withincreased mortality and critical care in pediatricpatients. Pediatr Crit Care Med. 2011;12(6):e233-e6.
- 22.Garcia PCR, Longhi F, Branco RG, et al. Ferritinlevels in children with severe sepsis and septicshock. Acta paediatrica. 2007;96(12):1829-31.
- 23.Bode SF, Lehmberg K, Maul-Pavicic A, et al.Recent advances in the diagnosis and treatment ofhemophagocytic lymphohistiocytosis. Arthritisresearch & therapy. 2012;14(3):1-12.
- 24.Arico M, Janka G, Fischer A, et al.Hemophagocytic lymphohistiocytosis. Report of 122 children from the International Registry. Leukemia.1996;10(2):197-203.
- 25.Tothova Z, Berliner N. Hemophagocyticsyndrome and critical illness: new insights intodiagnosis and management. J Intensive Care Med.2015;30(7):401-12.
- 26.Oruç N, Ozütemiz O, Osmanoğlu N, et al.Diagnostic value of serum procalcitonin indetermining the activity of inflammatory boweldisease. Turk J Gastroenterol: the official journal ofTurkish Society of Gastroenterology. 2009;20(1):9-12.
- 27.Floriańczyk B, editor Structure and diagnosticvalue of procalcitonin. Annales Universitatis MariaeCurie-Sklodowska Sectio D: Medicina; 2003.
- 28.Terpos E, Ntanasis Stathopoulos I, Elalamy I, etal. Hematological findings and complications ofCOVID 19. Am J Hematol. 2020.
- 29.Liu F, Li L, Xu M, et al. Prognostic value ofinterleukin-6, C-reactive protein, and procalcitoninin patients with COVID-19. J Clin Virol.2020:104370.
- 30.Huang C, Wang Y, Li X, et al. Clinical features ofpatients infected with 2019 novel coronavirus inWuhan, China. The lancet. 2020;395(10223):497-506.