Hemophagocytic Lymphohistiocytosis in Children

Abstract

Objective of the Paper: To present a clinical case of a 2.5-year girl hospitalised with severe pneumonia with associated hemophagocytic lymphohistiocytosis (HLH). Key Points. HLH is a syndrome, the clinical representation of which is a system sepsis-like inflammatory reaction; in the majority of cases, this condition is hardly diagnosed by paediatricians. Associated pancytopenia and hyperferritinaemia are of diagnostic importance. Here we present a clinical case of a 2.5-year girl who was hospitalised with severe pneumonia and associated HLH. As there is no common approach to the management of HLH, we summoned a board of doctors and decided to start steroid therapy (dexamethasone) and IV immunoglobulin (Octagam), and if there was no effect from therapy within a week, cytostatic agents would be added. The therapy was initiated promptly after macrophages had been found in bone marrow smear. Clinical and paraclinic improvements were noted on day 5, so aggressive immunosuppresion was not initiated. In a month there were no signs of organ dysfunction; the values normalised, and the child was discharged from the inpatient clinic. Conclusion. HLH is associated with high mortality, therefore, early diagnosis and therapy are critical. The specificity of this case is that we managed to promptly diagnose the condition, initiated treatment and achieved complete remission without aggressive immunosuppresion. Keywords: hemophagocytic lymphohistiocytosis, macrophages, pancytopenia.