Introduction/Objective. Hemophagocytic lymphohistiocytosis (HLH) is a severe
hyperinflammatory condition characterized by fever, splenomegaly and
cytopenias. Diagnosis of HLH requires at least five of the eight criteria
set by the Histiocyte Society and poses a significant challenge to
physicians. HLH-2004 criteria include measurement of plasma levels of
soluble receptor for interleukin-2 (sIL-2R), an invaluable tool in the
diagnosis of HLH, particularly because it can be measured swiftly and
inexpensively. Methods. We retrospectively analyzed medical records of 45
pediatric patients (28 boys and 17 girls, median age 8.1 years) who were
investigated for suspected HLH in University Children?s Hospital, Belgrade,
in the period 2012-2022. Results. Ten children were diagnosed with HLH,
while 35 did not have HLH. All ten HLH patients had secondary HLH: eight
suffered from infection or inflammatory condition, one from an autoimmune
disease, and one from malignancy. Level of sIL-2R was above the HLH-2004
cutoff value of 2400 IU/ml in 9/10 patients with HLH (sensitivity 90%) and
9/35 of patients who did not have HLH (specificity 74.2%). Conclusion.
Soluble IL-2 receptor measurement is valuable in children suspected to have
HLH. Sensitivity and specificity of this analysis can be further improved by
strict patient selection and a comprehensive diagnostic approach.