The Role of Pre-B-cell Colony- Enhancing Factor in Egyptian Children with Hemophagocytic Lymphohistiocytosis

Abstract Background Hemophagocytic lymphohistiocytosis (HLH) 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 Egyptian children with HLH. Subjects and Methods This is a cross-sectional study, recruited fifteen children with HLH from the Pediatric Hematology/Oncology unit. Patients underwent thorough clinical assessment lying stress on disease manifestation, classification, treatment and prognosis. Plasma concentration of PBEF was determined using an enzyme-linked immunosorbent assay. Results PBEF was significantly increase in patients group than control group. Four patients were classified as primary HLH, seven patients were 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 The elevated PBEF level and its correlation with the widely available biochemical markers for diagnosis of pediatric HLH indicating that it may be involved in its inflammatory process.