Specific Epstein-Barr Virus Infection Immune Response in Children with Hemophagocytic Lymphohistiocytosis and B cell Lymphoid Malignancies

Abstract Background Epstein Barr Virus Infection (EBV) is associated with significant morbidity and mortality in immunocompromised patients causing a wide spectrum of EBV associated immunopathologic conditions including lymphoproliferative disorders, hemophagocytic lymphohistiocytosis (HLH) and malignancy. The CD27/CD70 axis has a critical role in EBV immune surveillance. The clinical and immunological features of CD27 deficiency in patients presenting with B-cell malignancy and HLH remains to be identified Aim to evaluate of the frequency of CD27 deficiency in B cell lymphoid malignancy and HLH and to study its relation to EBV positivity. Methods A pilot study including twenty-one patients diagnosed with B cell lymphoid malignancy and HLH aged 4-10 years old (median 6 years) were initiated. EBV status was determined by detection of EBV nuclear antigen 1 (EBNA1) IgG by enzyme linked immune sorbent assay (ELISA) then quantification of CD27 on total lymphocytes and on CD3 by flow cytometry was performed. Immunoglobulin and HLH markers were reported from patients records in selected patients Results Patients with positive EBNA-1 IgG (n = 12) has significantly low percentage of CD27 expression on CD3 cells compared EBNA-1 negative ones (P = 0.028). CD27 expression did not differ according to EBV PCR status of the studied patients. The percentage of CD27 on lymphocytes showed a significant positive correlation with total leucocytic count and triglycerides level (p = 0.033 and 0.037 respectively) and a significant negative correlation with the fibrinogen level (p = 0.037) while absolute CD27 on lymphocytes was negatively correlated with age and fibrinogen level (p = 0.007, p < 0.001). Conclusion CD27 deficiency was found among chronic EBV infection in children with B cell lymphoid malignancy; However, further studies with higher sample size need to be pursued.