الفهرس 
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Abstract
Background: Hemophagocytic lymphohistiocytosis (HLH) is a clinical syndrome caused by a highly active but ineffective immune response, including impaired or absent function of natural killer cells and cytotoxic T cells, and the release of pro-inflammatory cytokines. Pre-B-cell colony-enhancing factor (PBEF) is an inflammatory cytokine involved in several inflammatory diseases and it has been identified to react with several cytokines involved in HLH.
Objective: we aimed to evaluate the role of PBEF as a diagnostic and prognostic marker in patients with HLH.
Subjects and Methods: The study was conducted at the pediatric hematology oncology unit, Ain Shams University. Fifteen patients were recruited and underwent through clinical assessment lying concentration on disease manifestation, classification, treatment and prognosis. Plasma concentration of PBEF was determined using an enzyme-linked immunosorbent assay.
Results: PBEF level was measured in the patients group, it was highly significantly increase for patients group than control group. Four patients were classified as primary HLH, seven patients were classified as secondary HLH and four patients had unknown classification due to waiting for genotyping. Seven patients of the study group were died and eight patients still alive. PBEF level showed a significant positive correlation with serum ferritin and triglycerides level and negative correlation with fibrinogen level.
Conclusion: An elevated PBEF level was observed in pediatric HLH, indicating that it may be involved in its inflammatory process. PBEF was correlated with the widely available biochemical markers for diagnosis of HLH.