الفهرس 
EBV VIRUSOnly 14 pages are availabe for public view
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Abstract
Epstein-Barr virus infection (EBV) is associated with significant morbidity and mortality in immunocompromised individuals causing a wide range of EBV associated immunopathologic conditions, including lymphoproliferative disorders (LPDs), hemophagocytic lymphohistiocytosis (HLH) and malignancy.The CD27/CD70 axis has a critical role in EBV immune surveillance.
The clinical and immunologic features of CD27 deficiency in severe EBV remain obscure because only a few patients have been identified to date.
This study was to evaluate the frequency of CD27 deficiency in EBV associated HLH and EBV associated B-cell lymphoid malignancies in paediatric population in comparison to non EBV associated HLH and malignancy.
This pilot study was conducted on 21 patients with B-cell lymphoid Malignancies and HLH attending the pediatric hematology/oncology clinic, children hospital, Ain Shams University, in the period from August 2021 till August 2022, they were 11 females and 10 males with age ranged from 2-15 year with median of 6 (4-10) years.
The aim of this study was to evaluate of the frequency of CD27 deficiency in EBV positive B cell lymphoid malignancies and EBV positive hemophagocytic lymphohistocytosis compared to EBV negative HLH and B cell lymphoid malignancy patients, and to evaluate of the clinical course and severity in CD27 deficient EBV associated HLH and lymphoid malignancies.
Patients diagnosed with B cell lymphoid malignancy according to National Comprehensive Cancer Network (NCCN) guidelines for pediatric lymphoma at their initial presentation. All eligible patients were subjected to the detailed history with data collected from the filing system.
Also laboratory studies were collected included EBV status by detecting EBV nuclear antigen 1 (EBV NA1) by ELISA. Lymph node biopsy pathology and Immunohistochemistry in case of lymphoma. Central nervous system (CNS) status by cerebrospinal fluid (CSF) analysis, and Flow cytometry for CD27 by cell staining and flow cytometry based phenotypic analysis of T cell will be performed according to standard flow cytometry methods.
There were 11 females (52.4%) and 10 males (47.6%) with age ranged from 2-15 years with median (IQR) of 6 (4-10) years.
We found that EBV PCR was significantly higher among patients with positive family history
In the relation between EBV PCR with disease subtypes, there was a significant higher frequency of EBV PCR positive patients in Hodgkin lymphoma compared to patients with non-Hodgkin lymphoma, ALL or HLH.
We found that a significant higher frequency of thrombocytopenia detected in patients with positive EBV PCR
In our study 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). However there was no significant difference in lymphocytes CD27 expression between EBV PCR positive and negative 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).
The titre of EBV-EBNA-1 Ig G had a significant negative correlation with total serum Ig G and Ig M (p<0.001,p =0.001 respectively) and a significant positive correlation with serum ferritin in HLH patients (p<0.001).
EBV PCR copy number shows a significant negative correlation with absolute lymphocytic count and hemoglobin level of the studied patients (p=0.019).
In the Relation between EBV PCR with imaging study, lymph nodes biopsy, we found that patients with positive EBV PCR has statistically higher percentage of positive CT chest and CT abdomen for lymph adenopathy findings compared to patients with negative EBV PCR, Also there was statistically significant difference among different diagnosis of studied patients as regards their EBV PCR status being more positive among patients with Hodgkin lymphoma.
Conclusion:
A preliminary data shows an underlying CD27 deficiency on CD3 may be associated with chronic and not recent EBV infection in children with B cell lymphoid malignancy.