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Hemophagocytic lymphohistiocytosis (HLH), a potentially fatal syndrome that occurs regardless of age, is classified into genetic and acquired forms. The exact mechanism is relatively unclear for the latter form. Acquired HLH has been associated with numerous diseases, including infections and rheumatologic disease. The key pathophysiology for HLH is hyperactivation and hyperproliferation of cytotoxic T cells and macrophages, as well as uncontrolled inflammatory cytokine production, which can result in end-organ damage and even death.
The main symptoms of HLH include persistent fever, pancytopenia, hepatosplenomegaly, and hemophagocytosis. Because most clinical features of HLH are nonspecific, establishing a diagnosis of HLH is time-consuming.
Because of this delayed recognition, the mortality of HLH was once as high as 95%. Currently, clinicians are seeking innovative methods for early diagnosis. However, there have been no studies to examine the role of PBEF in HLH.
Pre-B-cell colony-enhancing factor (PBEF) is an inflammatory cytokine involved in several inflammatory diseases. However, its role in HLH is unknown.
So in our study we aimed to evaluate the role of pre-B-cell colony enhancing factor (PBEF) as a diagnostic and prognostic marker in patients with HLH.
The study was conducted in the pediatric oncology unit, children hospital, Ain Shams University. Plasma was collected from 15 children with HLH who were diagnosed and treated in the unit, the concentration of plasma PBEF was determined using an enzyme-linked immunosorbent assay.
The data was collected by reviewing patient’s files and follow up sheets.
The age of the studied patients ranged from 6 to 120 months, they were 11 males and 4 females, all patients presented by splenomegaly as presenting symptom and 14 out of 15 presented by fever. Four patients had viral infection as a risk factor before presentation, three patients had malignancy and two patients had immune deficiency.
In our study, 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.
The disease progressed in 5 patients; 4 of them developed CNS infiltration and only one patient developed multiple reactivation.
Seven patients of the study group were died and eight patients still alive.
PBEF level was measured in the patient group, it was highly significantly increase for patients group than control group (P<0.001). The result of the control group was taken from work done by Gao et al. (2015).
We investigated the correlation between PBEF and laboratory findings of HLH and found that PBEF level showed a significant positive correlation with serum ferritin and triglycerides level and negative correlation with fibrin therefore in combination with known biomarkers. The determination of PBEF may be helpful for recognizing HLH.