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• W4385667514 abstract "Topic: 1. Acute lymphoblastic leukemia - Biology & Translational Research 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 lymphohistocytosis (HLH) and malignancy.The CD27/CD70 axis has a critical role in EBV immune surveillance. The clinical and immunological features of CD27 deficiency in severe EBV remain obscure because only few patients have been identified Aims: Evaluation 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 included twenty one patients diagnosed with B cell lymphoid malignancy and HLH aged 4-10 years old (median 6 years) were enrolled. History with emphasis on preexisting disease for malignancy like immunodeficiency or autoimmune disease, family history of similar conditions or sibling death, history of blood transfusion was taken then hematology. EBV status was determined by detection of EBV nuclear antigen 1 (EBNA1) IgG by enzyme linked immune sorbent assay (ELISA) and by detection of EBV DNA via real time quantitative polymerase chain reaction (PCR) then quantification of CD27 on total lymphocytes and on CD3 by flowcytometry was performed. 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). 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). Summary/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. The study will be pursued to get a larger sample size as a high frequency of EBV positivity is noted in this studied patients Keywords: B cell acute lymphoblastic leukemia, Hemophagocytic Lymphohistiocytosis (HLH), EBV, CD27" @default.
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• W4385667514 date "2023-08-01" @default.
• W4385667514 modified "2023-09-26" @default.
• W4385667514 title "PB1721: CD27 EXPRESSION IN CHILDREN WITH B CELL LYMPHOID MALIGNANCIES AND HEMOPHAGOCYTIC LYMPH HISTIOCYTOSIS IN RELATION TO EBV STATUS: A PILOT STUDY" @default.
• W4385667514 doi "https://doi.org/10.1097/01.hs9.0000973744.35882.81" @default.
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