Abstract
Background Hemophagocytic lymphohistiocytosis (HLH) is 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 proinflammatory 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 study the clinicoepidemiological characteristics of Egyptian children with HLH and to evaluate the role of PBEF as a diagnostic and prognostic marker in Egyptian children with HLH. Patients and methods This is a cross-sectional study that recruited fifteen children with HLH from the Pediatric Hematology/Oncology unit. There were 11 male and four female, and their median age (interquartile range) was 13 months. Patients underwent thorough clinical assessment, laying stress on disease manifestation, classification, treatment, and prognosis. Plasma concentration of PBEF was determined using an enzyme-linked immunosorbent assay. Results Four patients were classified as having primary HLH, seven patients were as having secondary HLH and four patients had unknown classification owing to waiting for genotyping. The risk factors before presentation were as follow: four had viral infection, three patients had malignancy, and two patients had immune deficiency before presentation. Seven patients of the study group died and eight patients were still alive. PBEF was significantly increased in the patient group than the control group. PBEF level showed a significant positive correlation with serum ferritin and triglycerides level and a negative correlation with fibrinogen level. Conclusion The elevated PBEF level and its correlation with the widely available biochemical markers for diagnosis of pediatric HLH indicates that it may be involved in its inflammatory process.
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